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Giant urethral calculus associated with a foreign body

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The authors report a case of a 50-year-old who complained of hardened mass in the anterior penis.

Authors: Gustavo Tomaz Franco *, Volnir D. Franco *, Camilo Idalino Vitergo, Ludmila Bertti Coelho, Bruno Peixoto Esteves.

Department of  Urology, Presbyterian Doctor Gordon Hospital of Rio Verde, Rio Verde, GO, Brazil.

Corresponding Author: Dr. Gustavo Tomaz Franco, Email address: [email protected] Dr. Volnir D. Franco, E-mail address: [email protected] of  Urology, Presbyterian Doctor Gordon Hospital of Rio Verde, Rio Verde, GO, Brazil.

Abstract
Background: Primary urethral calculi are rare in the West. Development is often associated with previous changes in urine flow, such  as diverticula, urethral stenosis and foreign bodies. The authors report a case of a 50-year-old who complained of hardened mass in the anterior penis. Urethrolithiasis diagnosis was made by radiographic imaging studies and a foreign rubber was found inside the giant stone after surgery.

 

Objectives
To report a rare case of giant urethrolithiasis by the introduction of a foreign body.

 

Methods
The case of a 50-year-old man suffering from mental development delay, negative history for diverticula and urethral stenosis is presented. Imaging tests revealed the presence of a giant urethral stone. A one-stage urethro-lithotomy approach was indicated.

 

Results
Postoperative follow up was satisfactory. The patient was discharged from the hospital two days after the surgery. Follow-up of 12 months showed no evidence of urethral stricture or recurrent stones.
Conclusion: To the best of our knowledge there are few published cases of urethral stones of the size we report. Management of urethral calculi varies according to their localization, their size and any associated urethral pathology. The treatment for an impacted, large calculus in urethra is urethro-lithotomy. An appropriate follow up is necessary for these cases.

 

Background 
The prevalence of primary urethral calculi varies according to the population studied, and the urethrolithiasis rates vary according to the region. This prevalence is uncommon in western countries and relatively frequent in Eastern countries, particularly in the Middle East and India. The urological literature about this subject is sparse, perhaps due to the rarity of cases, and in some cases the diagnosis is based solely on clinical findings. The urologist should be aware of the major risk factors associated with prior urethral changes, such as diverticula, stenosis areas and atypical findings which are often correlated with stone formation in urethral tube [1-5].
           The evaluation of these cases should take into account the patient´s age, clinical history, symptoms, urinalysis, urine culture, biochemical and haematological analysis and radiological imaging studies such as direct urinary system radiology and urinary ultrasonography. All this information will provide additional data for a better and more comprehensive approach to the patient [2-4]. Patients may occasionally be diagnosed with urethrolithiasis when a radiologic imaging study of the pelvis is performed. [6-9].
         In the case reported below, the aetiological factor of stone formation – a foreign body – was discovered only after surgery, although the clinical history and radiologic studies suggested the possibility of a foreign body in the urethral tube. Urinary calculus formation in the urethral tube is seldom seen and is usually found in men with urethral stricture or diverticulum [10,11,12]. Most of calculi of the urinary system are located in the upper region of the  urinary system and stone formation usually occurs when  soluble material (e.g., calcium) supersaturates the urine and begins the process of crystal formation. Urethrolithiasis  represents less than 1% of all lithiasis [1,3,10]. The calculi are usually formed elsewhere and get stuck in the proximal urethra, but they may rarely be formed primarily in the urethra [1,2,3,10].
          Urethral stones are seldom encountered  in urological practice and the  published studies  consist of small series and several case reports. Giant urethral calculi are even rarer and to the best of our knowledge there are few published cases about urethral stones of the size we report.  Most urethral calculi are originated from bladder migration. Calculi in the urethra can be mainly formed in the proximal urethra and are primarily related to stenosis or urethral diverticulum. Most calculi occur in male urethral tube and they are extremely rare in females [3,12,13,14,15].

 

Case report
A 50-year-old man was admitted at the emergency service of Presbyterian Doctor Gordon Hospital of Rio Verde in January 2010 with clinical findings of oedema and sharp pain in the penis and scrotum, for the past 2 months. He also complained about a hardened mass in the anterior penis. The patient’s past medical history was positive for hepatitis type B and for mental development delay by birth. He did not show any other significant medical problems and past medical history was also negative for urethral diverticulum and for urethral stenosis. The patient showed a history of dysuria with no associated fever. He had no episodes of haematuria.
         The physical examination revealed an increase of penis volume with inflammatory signs and a small leakage (fistula) of urine at the peno-scrotal junction. There was only mild discomfort. He had also report normal urinary flow. He had no fever or any other relevant systemic diseases. However, the scrotum revealed marked oedema with no purulent lesions, and a crackle around the penis on the ventral surface near the glans was noted. He was previously treated with ciprofloxacin for urinary tract infection.
            Plain radiographs of the pelvis showed the image of a giant urethral calculus (Figure 1).

 

Figure 1. Several steps of urethrocystography of the pelvis showed a large calculus in the  urethra. 

 

Plain X-ray and ultrasound scans of the kidney, ureter and bladder area were all normal. General physical examination did not reveal any abnormality. The possibility of a foreign body in the urethra was considered. Routine metabolic work-up and renal function tests were normal. Urine culture did not show any growth. Levels of serum parathyroid hormone and serum calcium were within normal range. Digital rectal examination did not detect any abnormality. A one-stage urethro-lithotomy approach was indicated and the patient underwent an open surgery.
            The following steps were performed during the surgery: 1- Circular incision of the foreskin around one cm below the glans. 2- Foreskin degloving release down to the base of the penis 3- Incision at three longitudinal urethral points corresponding to locations of the urethral stones. 4- Urethro-lithotomy  (Figures 2 and 3).

 

Figures 2 and 3 – Stone removal through urethra-lithotomy.  

 

5- uretheral closure using Vycril® 4.0 with running suture dual layer was performed. 6- Excess of preputial skin was resected and cutaneous sutures were placed across the mucosa circumference of penis. 7- Urethral catheterization was performed with a foley catheter number 18 and a suprapubic cystostomy was finished by a trocar and a foley catheter number 16 was left. After the surgery, a rectangular rubber fragment was found inside the urethral stones, which represented  the source of  calculus development (Figures 4 and 5).

 

Figure 4 – Calculus macroscopic aspect

 
Figure 5 – Presence of three urethral stones, the largest measuring about 5 cm in the greatest diameter.

 

           Postoperative follow up was satisfactory. The patient was discharged from the hospital two days after surgery. The duration of catheterization was 15 days for urethral catheter and 21 days for cystostomy. The patient had no complications in this period. On the 30th day, he returned to the ambulatory clinic with no urinary complaint. On the 90th day after the surgery, he was submitted to  urethrocystography, which was normal ( Figures 6, 7 and 8).

 

Figure 6 – Plain X-ray of the pelvis showing a large calculus in the urethra. 

 

Figure 7- Urethrocystography of the pelvis showing  three  suggestive images  of urethra calculi. 

 
Figure 8- Normal urethrocystography  90 days  after surgery.

Follow-up at 12 months showed no evidence of urethral stricture or recurrent stones.

 

Discussion
Urethral calculi are rarely found in urological practice and published studies  consist of small series and several case reports. Most urinary stones are located in the upper urinary tract so calculi in the urethra are especially rare (around 1%) [1,3,10]. In most cases, the   stones formed in the high urinary tract  are migrated to the urethra and get  stuck in this area. Theoretically, the caliber of the urethra of the adult (30 F) allows the passage of stones with a diameter less than or equal to 10 mm. However, when there is stenosis or a large stone, the anatomic conditions become uncertain to allow their free progression [2]. There is also well established association between the diagnosis of calculi of the urethra with local diverticula, chronic urinary infection, obstruction of variable etiology and foreign bodies [1,6,7], and there are indications that the dietary habits and hygienic conditions are involved in the pathogenesis of the disease [4].
 Depending on the origin, urethral calculi are classified as primary or secondary (due to migration) [5,6,7]. Secondary stones are more common than primary ones and the stones migrate from the upper urinary tract [5,6,7,8]. Urethral calculi are usually small [5,6].  Giant calculi in the urethra are rare, but in some specific groups of patients they could be developed around foreign bodies inserted in the urethral tube. As the urinary symptoms are early, which often follow the foreign body´s insertion, the diagnosis is generally early as well [3]. However in this case this patient looked for medical help tardily, perhaps due to mental development delay.
              The progression of urethral lithiasis depends on the patient´s reaction to the foreign element and this is determined by the type of the inserted material, its duration in the patient´s body, associated infection and the individual´s immunological response [7].
               Most urethral calculi occur in the male [4]. Urethrolithiasis prevalence is much higher in men, due to the increased length of the urethral canal, and the age varies from 3- to 81- year- old individuals [4,5,22]. The group most affected by the  insertion of foreign bodies in the urinary tract are the  young adults, probably caused by the exercise of masturbatory practices [5], the psychiatric patients [3,5,6], patients  with cognitive impairment and  alcoholic patients [6].
                 Urethral calculus can be completely asymptomatic or may be accompanied by one of the following symptoms: perineal or penile pain, increased urinary frequency, urgency, decreased urinary stream, haematuria and / or urethral discharge due to infection. The patient can show signs of acute urinary retention or insignificant history. The diagnosis is based on clinical history and relevant investigations [5-10]. The appropriate management in the presence of urethral calculus will vary depending on the location and size of the stones and the presence or absence of associated diseases in the urethral canal. Retrograde manipulation for the urinary bladder followed by litholapaxy or lithotripsy is an appropriate procedure for posterior urethral calculus [14-20].
Stones in the anterior urethra can be treated with instillation of lidocaine jelly 2% or ventral meatotomy according to their location. Giant urethral calculus should be treated with open surgery. In addition, if the urethra has a stenosis or has been damaged by previous attempts to extract a stone, urethroplasty is preferred [3,23,24]. In the immediate postoperative period the patient can develop an urinary tract infection, pain, fistula and urinary retention. In the late postoperative, patients can develop urethral stenosis [7,10,14,21,22,25).

 

Conclusions
There are few published cases about urethral stones the size we report. Urethral primary stones are rare and correspond to less than 1% of urinary tract calculus. Management of urethral calculi varied according to the site, size and associated urethral pathology. The diagnosis can be done by radiologic imaging studies. The choice of treatment for an impacted, large calculus in urethra is urethro-lithotomy with stone removal. One stage procedure (urethro-lithotomy) was indicated.
Retrograde manipulation for the urinary bladder followed by litholapaxy or lithotripsy is an appropriate procedure for a posterior urethral calculus. For the anterior urethra, meatotomy or urethrotomy is generally used. Endoscopic treatment can be performed as well, but sometimes the urothelium may obscure the foreign body.
In this case report, the patient showed dysuria only. Urethral stricture and  diverticulum were not detected. Follow-up of 12 months showed no evidence of urethral stricture or recurrent stones. An appropriate follow up is necessary for these cases.

 

References
1.  Hassan I, Mahammed I. Urethral calculi: a review. East Afr Med J 1993;70:523–5.
2.  Hemal AK, Sharma SK. Male urethral calculi. Urol Int 1991;46:334-7.
3.  Noble JG, Chapple CR. Formation of a urethral calculus around an unusual foreign body. Br J Urol 1993;72:2489.
4.  Koga S, Arakaki Y, Matsuoka M, Ohyama C. Urethral calculi. Br J Urol 1990;65:288–9.
5.  Pinto AC, Patrício PS, De Luccas V, Pinto AFC. Corpo estranho intravesical: revisão de 10 anos. J Bras Urol 1997;23:1–4.
6.  Ali Khan S, Kaiser CW, Dailey B, Krane R. Unusual foreign body in the urethra. Urol Int 1984; 39:184–6.
7.  Painter MR, Borski AA, Trevino GS, Clark WE. Urethral reacion to foreign objects. J Urol 1971; 106:227–30.
8.   Win T. Giant urethral calculus. Singapore MedJ1994;35:4145.
9.   Amin HA. Urethral calculi. Br J Urol 1973;45:192–9.
10. Drach GW: Urinary lithiasis: Etiology, diagnosis and medical management. In Walsh P, Retik A, Stamey, Vaughan D. Eds. Campbell’s Urology, 6th edn. Philadelphia:W.B. Saunders, 21442145, 1992.
11.   Fernandez Fernandez A, Santamaria Roa A, Soria Ruiz S, Gil Fabra J, Gil Paraiso A, Otero Mauricio G: Giant urethral lithiasis. Arch Esp Urol. 46: 914-6, 1993.
12.  Gonzalvo Perez V, Botella Almodovar R, Canto Faubel E, Gasso Matoses M, Llopis Guixot B, Polo Peris A: Urethral diverticulum complicated with giant lithiasis. Actas Urol Esp. 22: 250-252, 1998.
13.  Suzuki Y, Ishigooka M, Hayami S, Nakada T, Mitobe K: A case of primary giant calculus in female urethra. Int Urol Nephrol. 29: 237-239, 1997.
14.  Sharfi AR: Presentation and management of urethral calculi. Br J Urol 68: 271-275, 1991.
15.  Hemal AK, Sharma SK: Male urethral calculi. Urol Int. 46: 334-339, 1991.
16.  Koga S, Arakaki Y, Matsuoka M, Ohyama C: Urethral calculi. Br J Urol 65: 288-92, 1990.
17.  Paulk SC, Khan AU, Makek RS, Greene LF: Urethral calculi. Urology 16: 436-440, 1976.
18.  Bridges CH, Belville WD, Buck AS, Dresner ML: Urethral calculi. J Urol. 128: 1036-1037, 1982.
19.  Kessler A, Rosenberg HK, Smoyer WE, Blyth B: Urethral stones: US for identification in boys with hematuria and dysuria. Radiology. 185: 767-768, 1992.
20.  Selli C, Barbagli G, Carini M, Lenzi R, Masini G: Treatment of male urethral calculi. J Urol 132: 37-42, 1984.
21.  Durazi MH, Samiei MR: Ultrasonic fragmentation in the treatment of male urethral calculi. Br J Urol 62: 443446, 1988.
22.  Walker BR, Hamilton BD: Urethral calculi managed with transurethral Holmium laser ablation. J Pediatr Surg. 36: E16, 2001.
23.  Suarez GM: Re: Treatment of male urethral calculi. J Urol. 133: 292, 1985.
24.   Kamal BA, Anikwe RM, Darawani H, Hashish M, Taha SA: Urethral calculi: Presentation and management. BJU International. 93: 549-552, 2004.
25.  Larkin GL, Weber JE: Giant urethral calculus: A rare cause of acute urinary retention. J Emerg Med. 14: 707709, 1996.

 

Date added to bjui.org: 10/05/2012 


DOI: 10.1002/BJUIw-2011-018-web

 

Second-degree burn after shock wave lithotripsy: an unusual complication

/1 Comment/by

We present an unreported complication of a second-degree burn sustained by a 62-year-old male who underwent shockwave lithotripsy for two renal calculi. 

 

Authors: Sriram Rangarajan1, Hossein Mirheydar1, Roger L. Sur1,2

1. UC San Diego Health Sciences, Department of Surgery, Division of Urology; San Diego, CA
2. VA San Diego Medical Center, San Diego, CA

Corresponding Author: Roger L. Sur, MD, UC San Diego Health Sciences, Division of Urology, 200 W Arbor Dr. #8897, San Diego, CA 92103-8897. T: 619-543-2630   E-mail: [email protected]

Abstract
We present an unreported complication of a second-degree burn sustained by a 62-year-old male who underwent shockwave lithotripsy for two renal calculi.  The patient was treated conservatively for his burn injury but did require several ancillary procedures to render him stone free.  We speculate that the number of shocks and bubbles associated with an unapproved coupling medium for this particular lithotripter may have led to this highly unusual adverse event.

 

Introduction
 
Shockwave lithotripsy is considered a minimally invasive procedure for upper tract urinary stone disease with the obvious benefit of minimal side effects and rare adverse events.  These advantages have helped evolve it into a common surgical treatment modality especially due to its safety profile.  We report an unusual adverse event following shockwave lithotripsy involving a second degree skin burn at the coupling site.

 

Case Report
 
A 62-year-old Caucasian male presented with gross hematuria and left-flank pain. He had been seen in the emergency department 18 months prior for left-flank pain associated with two calcifications (a 9 mm midcalyceal calculus and an 8 mm lower pole calculus).  The patient’s past medical history was unremarkable. His medications included allopurinol, 300 mg, and aspirin, 81 mg, both of which he had discontinued one week prior to procedure. Physical examination revealed mild left costovertebral angle tenderness.  Computerized tomography (CT) urogram of the abdomen and pelvis demonstrated the two previously noted renal calculi  (10 mm,1500 HU in the left calyx and 9mm,1000 HU in the left calyx ).  Cystoscopy and cytological examination of two urine specimens did not reveal any abnormality.
The patient opted for shockwave lithotripsy (SWL). An electromagnetic lithotripter (Medispec EM1000, Germantown, MD), which our institution leases from Medispec, was used, and 4000 shocks (2000 to each calculus) were delivered at 90 shocks/minute after gradually increasing the power to 20kV. The Medispec technician did not have the standard coupling gel recommended by Medispec and instead used ultrasound gel available from the hospital.  In the recovery unit, the entire skin overlying the area where the gel had been placed was noted to be moderately erythematous with 3-4 cm central area of subcutaneous hemorrhage (figure 1).

 

Figure 1. demonstrates the immediate post-operative changes noted in the recovery unit: erythema and central region of petechiae
The patient returned on postoperative day 1 with significantly increased erythema and the development of large blisters (figure 2).  He also noted some abdominal pain, gross hematuria and passage of small fragments. The burn surgery service was consulted. A second-degree burn was confirmed, and local debridement was performed along with application of Collegnase, polymyxin B, xeroform, and gauze dressings.

 

Figure 2 demonstrates development of marked erythema and bullae on post-operative day 1

 

 

The patient returned again on postoperative day 5 complaining of left-flank pain and vomiting. The  wound remained erythematous with denuded skin. A noncontrast CT of his abdomen and pelvis CT  demonstrated an interval decrease in stone burden but multiple fragmented calculi (900HU) in the lower calyx as well as at least two adjacent 6 mm calculi in the proximal ureter (HU 1000). The patient consented to ureteroscopy with Ho:YAG laser lithotripsy.  During the left ureteroscopy with Ho:YAG laser lithotripsy and ureteric stenting, the ureteric calculi migrated into the kidney and one large calculus was fragmented.  A significant amount of mucous material in the urine precluded good visualization. The indwelling stent was removed 6 days later at outpatient cystoscopy.
Renal ultrasound performed 5 weeks later demonstrated a moderate left hydronephrosis and CT of the abdomen and pelvis revealed a 2 cm column of distal ureteral calculi. Subsequent ureteroscopy was aborted because of an iatrogenic extraperitoneal bladder perforation during dilation of an unexpected bulbar urethral stricture encountered during initial cystoscopy.  After documenting healing of the bladder, left ureteroscopy with Ho:YAG laser lithotripsy and ureteraic stenting was performed 10 days later. This rendered the patient stone free, as confirmed by direct visualization. Outpatient cystoscopic stent removal was performed 5 days later. He was asymptomatic two months afterwards with normal voiding and a  normal appearance on renal ultrasound.

 

Discussion
 
Complications following SWL are rare but include renal hematoma 1-13 % [1-3], steinstrasse 2-10% [4], acute renal injury, and theoretically, chronic renal injury such as a risk of hypertension [5].  To our knowledge this is the first case documenting a second-degree skin burn following SWL treatment.  There is no obvious explanation for this unusual adverse event, but some facts specific to this individual case deserve mention.  Firstly, we acknowledge that 4000 shocks might be considered marginally high. However, this has been routinely delivered with this particular lithotriptor in appropriate cases where calculi were in different renal locations, and this adverse event had never been experienced previously.  The two stones was located in different regions of the kidney and required 2000 shocks to adequately fragment under fluoroscopy.  It is tempting to surmise that a fewer shocks would have prevented this outcome.
We also note that conventional ultrasound gel was used in lieu of standard coupling gel approved for use with the Medispec EM1000 lithotripter.  The Medispec-recommended coupling medium is Lithoclear gel (Sonotech, Bellingham, WA). It has a density of 1.013 g/cc and is made of water-based couplants with a general composition of >80% water, 5 to 15% propylene glycol, 1 to 5 % glycerine, an acrylic polymer and a cosmetic-grade preservative.  The Lithoclear gel is filled and reportedly packaged under tighter controls than ultrasound gel to ensure that the containers are absolutely free of micro- and macrobubbles that would cause reflection or scattering of shock wave acoustics during SWL [6].  Anecdotally, it has higher viscosity than ultrasound gel. Higher viscosity may result in lower tendency to form bubbles [7].  The ideal medium would mimic the viscosity of water to minimize loss of energy but would be viscous enough that it would not run off the patient during the procedure.  The medium should also be devoid of air bubbles, as they are thought to further attenuate the energy transmission to the patient. Just as cavitation functions to fragment calculi during lithotripsy, some have proposed that cavitation of bubbles within the coupling medium is responsible for skin pain [7].  Could the skin burn be attributed to cavitation of excessive bubbles within the nonapproved ultrasound gel? Ultrasound gel is routinely used as a coupling agent in some centers, though ensuring the presence of bubbles is minimized is commonly advocated [8].  We can only speculate that excessive cavitation from the coupling medium associated with an elevated number of delivered shocks may have been sufficient to generate a thermal injury.  Another explanation is heat generated from the water-filled cushion, though there is no plausible reason to think this is possible.  The specific lithotripter used for this case was evaluated by the maker, Medispec, and no mechanical abnormalities were discovered.

 

References

 

1. Evan AP, Willis LR (2007). Extracorporeal shock wave lithotripsy: complications. In: Smith AD, Badlani G, Bagley D, eds. Smith’s Textbook of Endourology. 2nd ed. Hamilton, ON, BC Decker, 2007; chap 41: 353–65.
2. Orozco Farinas R, Iglesias Prieto JI, Massarrah Halabi J, Mancebo Gómez JM, Perez-Castro Ellendt E. Renal hematoma after extracorporeal shockwave lithotripsy in a series of 324 consecutive sessions with the DOLI-S lithotripter: incidents, characteristics, multifactorial analysis and review. Arch Espan Urol. 2008; 61: 889–914.
3. Mobley TB. Low energy lithotripsy with the lithostar: treatment results with 19,962 renal and ureteral stones. J Urol. 1993; 149: 1419–24.
4. Madbouly K, Sheir KZ, Elsobky E, Eraky I, Kenawy M. Risk factors for the formation of a steinstrasse after extracorporeal shock wave lithotripsy: a statistical model. J Urol. 2002; 167: 1239–42.
5. Lingeman JE, McAteer JA, Assimos DG, Baxley J, Kahn RI, Krambeck A, Matlaga BR, Penson D, Preminger GM, Zhong P. White Paper: Current Perspective on Adverse Effects in Shock Wave Lithotripsy. American Urological Association Education and Research, 2009.
6. Email communication with Sonotech.
7. Heidenreich A, Bonfig R, Wilbert DM, Engelmann UH. Painless ESWL by cutaneous administration of Vaseline. Urologe A. 1995 Jul;34(4):343-7. German
8. Cartledge JJ, CrossWR, Lloyd SN, Joyce AD. The efficacy of a range of contact media as coupling agents in extracorporeal shockwave lithotripsy. BJU Int. 2001; 88: 321–4.

 

Date added to bjui.org: 25/04/2012 


DOI: 10.1002/BJUIw-2012-006-web

 

Duplex Megaureter Misdiagnosed as a Hydrosalpinx on Ultrasound

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We present a young female patient who presented with recurrent urinary tract infections, which investigation revealed to be due to megaureter initially thought to be a hydrosalpinx. 

Authors: Kass-Iliyya A1; Beck R1; Kass-Iliyya G2; Iacovou J1

1. Department of Urology, Great Western Hospital, Swindon, UK
2. Department of Urology, Knappschaft Hospital, Sulzbach, Germany

Corresponding Author: Antoine Kass-Iliyya, Urology Department, Great Western Hospital, Swindon, UK, SN3 6BB. Email: [email protected]

 

Introduction
Ureteric duplication refers to the presence of two complete and separate ipsilateral ureters. The kidney on the affected side may have a duplicated pelvicalyceal system or there may be two separate kidneys.
The reported incidence of ureteral duplication is 1 in 125, or 0.8%.
Unilateral duplication occurs about six times more often than bilateral duplication, with the right and left sides being involved about equally. In duplicated ureters, the two orifices are characteristically inverted in relation to the collecting systems they drain. The orifice to the lower pole ureter occupies the more cranial and lateral position, and that of the upper pole ureter has a caudal and medial position (the Weigert-Meyer law). [1]
No specific clinical signs are associated with ureteric anomalies. Most children present with an abnormal finding on routine prenatal ultrasound before associated symptoms or infection develops. Some patients present with urinary tract infections (UTIs ), an abdominal mass or haematuria. Children with primary megaureters may also present with cyclic abdominal  or flank pain, or, less commonly, in an acute pain crisis. [2]

 

Case report
A 16 year-old girl was initially referred to the Paediatric team with recurrent UTIs and chronic pelvic pain causing many school absences. Ultrasound scan revealed a hypoechoic area on the right side of the pelvis, thought to be a right-sided hydrosalpinx. (Figure 1)

 

Figure 1. Right sided megaureter (MGU) (green arrow), initially thought to be a hydrosalpinx.110x96mm (72 x 72 DPI)

 

A diagnostic laparoscopy and cystoscopy performed after failure of conservative measures to control the pain revealed a normal uterus, ovaries, fallopian tubes and an inflamed bladder. An  intravenous urogram (IVU) demonstrated a dilated right ureter, reduced number of calyces in the right kidney, and normal left kidney and ureter. (Figure 2)

 

Figure 2. IVU showing the typical drooping lily sign, as a result of infero-lateral displacement ofthe lower pole calyces by an obstructed hydronephrotic upper pole. (green arrow). 129x176mm (72 x 72 DPI)

 

More detailed investigation with contrast enhanced magnetic resonance imaging (MRI) demonstrated complete ureteric duplication with a widely distended upper moiety pelvic ureter opening in close proximity to the bladder neck, and a moderately distended lower moiety ureter opening superio-laterally to its twin ureter. (Figure 3)

 

Figure 3. MRI picture of the right kidney, showing complete ureteral duplication with a hydronephrotic upper moiety and a corresponding megaureter (red arrows), and a normal lower moiety and mildly dilated corresponding ureter. (green arrows). 57x89mm (72 x 72 DPI)

 

The distended and tortuous ectopic ureter was the source of the recurrent urinary tract infections, and pelvic pain.
On reviewing the ultrasound images of the right kidney in retrospect, the two moieties could be identified separately as shown in Figure 4.

 

Figure 4. Ultrasonic image of the right kidney showing separate collecting systems/moieties. (green arrow) 95x97mm (72 x 72 DPI)

 

Furthermore, on reviewing MAG3 renogram images also performed for functional assessment, a truncated looking right kidney was demonstrated, indicating little or no function in the upper obstructed moiety. (Figure 5)

 

Figure 5. (MAG-3) renal scintigraphy, showing a truncated looking right kidney, indicating poor function of the upper moiety. (green arrow) 41x69mm (72 x 72 DPI)

 

Discussion 
The term megaureter (MGU) is simply a descriptive name that conveys the picture of a dilated ureter. It implies no particular unifying pathophysiologic principles but merely groups together a spectrum of anomalies associated with increased ureteric diameter, usually wider than 7 to 8 mm. [1]
MGU can be associated with complete ureteric duplication –as demonstrated in this case. The latter has a propensity for vesicoureteric reflux into the lower pole and obstruction of the upper pole. This can be a source of recurrent urinary tract infections and repeated hospitalization. [2]

 

Investigations of duplex ureters 
Postnatal abdominal ultrasonography is the best initial screening study. Disparate hydronephrosis in the upper and lower pole of a kidney suggests ureteral duplication, especially with upper pole dilation associated with an obstructed or ectopic ureter or with a ureterocele. [3,4]
IVU findings are mostly diagnostic. Difficulty may arise when function is poor or absent in one of the moieties. [3] An obstructed, poorly functioning upper pole moiety of a duplex kidney –as demonstrated in this case- can exert a mass effect on the lower pole collecting system which causes lateral displacement of the lower pole moiety giving rise to the so called drooping lily sign; the appearance of the lower pole collecting system is reminiscent of a lily flower that is wilting or drooping. (Figure 2)
Magnetic resonance (MR) urography may be used as the primary diagnostic method for assessing a duplex ectopic ureter, as well as the complications associated with duplex kidneys, however it is rarely the initial study of choice due to cost considerations. [5,6]
Mercaptoacetyltriglycine (MAG-3) renal scintigraphy provides information on segmental renal function, allowing comparison of the upper pole moiety to the lower. It may aid in the determination of salvageability and selection of operative technique. [7]

 

Management

 

With salvageable renal function, a number of surgical options are available, depending on the cause, the degree of dilatation, and whether one or both ureters are abnormal.
When only one ureter of a duplex system needs to be addressed options include: ureteroureterostomy and ureteropyelostomy. When both ureters require correction, they can be mobilized within the bladder as a common sheath and reimplanted by a standard technique to complete the repair. [1]
In cases in which there is no function, as represented in this case, excision of the MGU is performed in concert with heminephrectomy, which could be performed laparoscopically in selected centers. [1]
This girl underwent laparoscopic heminephrectomy and had a good recovery.

 

Conclusion
 
Complete duplex ureters should always be considered in any child, teenage or adult with intractable urinary tract infections.
Ultrasonography and excretory urography (IVP) are usually diagnostic; however, renal scintigraphy and contrast enhanced MRI greatly aid the diagnosis in cases of uncertainty or when there is a poorly functioning moiety.
Radiologists can occasionally miss some subtle features of a duplex system, which reiterates the importance of clinical correlation and more involvement in radiological interpretation by urologists.

 

References
[1] Wein AJ, Kavoussi LR, Novick AC, et al, eds. Campbell-Walsh urology, 9th ed. Philadelphia: WB Saunders; 2007:3413-3481
[2] Gatti J, Murphy P, Williams J, et al. Ureteral Duplication, Ureteral Ectopia, and Ureterocele. Emedicine.medscape.com/article/1017202
 [3] Khan AN, Chandramohan M, MacDonald S, et al. Duplicated Collecting System Imaging. Emedicine.medscape.com/article/378075
[4] Adiego B, Martinez-Ten P, Perez-Pedregosa J, et al. Antenatally diagnosed renal duplex anomalies: sonographic features and long-term postnatal outcome. J Ultrasound Med. Jun 2011;30(60):809-15.
[5] Avni FE, Nicaise N, Hall M, et al. The role of MR imaging for the assessment of complicated duplex kidneys in children: preliminary report. Pediatr Radiol. Apr 2001;31(4):215-23.
[6] Yanagisawa N, Yajima M, Takahara T, et al. Diagnostic magnetic resoncance-urography in an infant girl with an ectopic ureter associated with a poorly functioning segment of a duplicated collecting system. Int J Urol. May 1997;4(3):314-7.
[7] Wu F, Snow B, Taylor A Jr. Potential pitfall of DMSA scintigraphy in patients with ureteral duplication. J Nucl Med. Jul 1986;27(7):1154-6.

Date added to bjui.org: 24/04/2012


DOI: 10.1002/BJUIw-2011-148-web

Three Dimensional Computerized Tomography Imaging of an Incidentally Detected Inguinoscrotal Bladder Hernia

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We report a case of massive inguinoscrotal bladder hernia confirmed with three-dimensional CT imaging. 

Authors: RIFAIOGLU Murat Mehmet1; BAYAROGULLARI Hanefi2; AKKUCUK Seckin3; AYDOGAN Akin3; DAVARCI Mursel1; DEMİRBAS Onur1; YETIM Ibrahim3

1. Mustafa Kemal University, Faculty of Medicine, Department of Urology, Serinyol, Antakya, TURKEY.
2. Mustafa Kemal University, Faculty of Medicine, Department of Radiology, Serinyol, Antakya, TURKEY.
3. Mustafa Kemal University, Faculty of Medicine, Department of General Surgery, Serinyol, Antakya, TURKEY.

Corresponding Author: Murat M RIFAIOGLU, MD, Mustafa Kemal University, Faculty of Medicine, Department of Urology, 31005, Serinyol, Antakya, Hatay, TURKEY.  Tel: +90 326 229 10 00-3317   E-mail: [email protected]

 

Abstract
Bladder related inguinal hernias are seen in less than 4% of cases. Computed tomography (CT) appears to be the best choice to outline the details of herniation. We report a case of massive inguinoscrotal bladder hernia confirmed with three-dimensional CT imaging. A 55-year-old man presented complaining of lower urinary symptoms and inguinal discomfort. Ultrasonography of the testes revealed a large cystic mass in the right hemiscrotum that was greater than 9 cm in size. Three-dimensional CT scanning revealed deviation of the right lateral wall of the urinary bladder into the right inguinal canal and scrotum. The bladder was reduced into the abdominal cavity without complication. The Prolone Hernia System repair that was described by Gilbert was used for the inguinal hernia repair and the patient had an uneventful postoperative course. Three-dimensional CT is the best imaging technique to reveal bladder hernia and also has the advantage of being non invasive.

 

Introduction
 
Bladder related inguinal hernias are seen in less than 4% of cases [1, 2]. Massive scrotal cystoceles are very rare, with less than 30 cases published in the literature. Early diagnosis with radiologic imaging is important to avoid complications during surgery. Computed tomography (CT) appears to be the best choice to outline details of the herniation [3]. Here we report a case of massive inguinoscrotal bladder hernia confirmed with three-dimensional CT imaging in a 55-year-old man.

 

Case Report 
A 55-year-old man presented complaining of low urinary tract symptoms, with  urgency and inguinal discomfort over a 10 month period. Physical examination revealed a left lumbar incision and three laparoscopic port incision scars from a left simple nephrectomy and laparoscopic cholecystectomy, ten years and two months ago, respectively. His abdomen was soft without any palpable masses, and no flank tenderness was evident. A 13-cm right sided scrotal mass was present that was soft, non tender, did not transilluminate, was without bowel sounds, and was not reducible. The right testis could not be palpated. The patient’s serum blood urea nitrogen was 30 mg/dL, with a baseline creatinine of 1.7 mg/dL. His body mass index was 33.1 kg/m2, and his PSA was 0.25 ng/mL. His prostate volume was 21.3 cc on abdominal ultrasonography. On uroflowmetry, his maximum flow rate (Qmax) was 15 mL/s and the voided volume was 320 cc. The uroflow curve was bell-shaped. Ultrasound scan of his bladder revealed no residual urine. The results of laboratory tests, including urinalysis and urine cultures, were normal. Urinary tract ultrasonography revealed a 10.5 mm stone  in the lower pole of the right kidney. A non-contrast CT was performed to investigate whether any other renal calculi were present. The scan revealed right renal calculi and that the patient’s right bladder wall was herniated into the scrotum. Ultrasound of the testes revealed a large cystic mass in the right hemiscrotum that was greater than 9 cm in size, without any solid portion or vascularity, and no definite connection to the pelvic cavity (Fig. 1).

 

Figure 1. Axial (a) and sagittal (b) plane ultrasonography examination of the inguinal region showed a cystic mass.

 

Three-dimensional CT was performed with intravenous contrast enhancement and revealed a solitary right kidney, lateral deviation of the right lateral wall of the urinary bladder, and a fluid-filled structure in the right inguinal canal and scrotum. The volume of the pelvic component of the bladder was measured at 255 ml; the scrotal component volume was 252 ml (Fig. 2).

 

Figure 2. Multislice spiral computerized tomography (MSCT) (a-c) and three-dimensional MSCT images (d-f) which demonstrated subtotal prolapse of the whole bladder into the scrotum.

 

On three-dimensional CT, the craniocaudal diameter of the internal inguinal ring was measured at 26 mm; transverse diameter was measured at 25 mm..
After informed consent was taken, a midline laparotomy performed under general anesthesia identified right inguinoscrotal herniation of the bladder. The bladder was dissected from the inguinal canal and intraoperative inspection demonstrated no evidence of bladder necrosis or hernia neck. The bladder was reduced into the abdominal cavity without complication. The Prolone Hernia System repair that was described by Gilbert was used for the inguinal hernia repair and the patient had an uneventful postoperative course (Fig. 3)[4].

 

Figure 3. Intraoperative appearance of the herniated bladder (arrow) (a). Image of the herniated bladder after reduction (b). Using Prolone Hernia System repair in primary inguinal hernia (B: bladder, HB: herniated bladder, M: mesh) 

 

A follow-up cystogram on day 15 demonstrated no recurrent hernia (Fig. 4).

 

Figure 4. Cystogram demonstrated no recurrent hernia

 

After 1 month of Tamsulosin therapy, the patient had normal urinary voiding with moderate frequency and no evidence of hernia recurrence. He will require follow up for his renal calculus.

 

Discussion
The bladder is involved in less than 4% of all inguinal hernias, and most cases are not diagnosed prior to surgical repair [1-3, 5]. Most bladder hernias are direct, with a 70% male predominance, and most cases occur on the right side [6]. Bladder hernias are anatomically classified into paraperitoneal, which is the most frequently occurring type, intraperitoneal and extraperitoneal [7, 8]. Our case was a paraperitoneal indirect hernia where the extraperitoneal portion of the bladder lies along medial to the right sided hernial sac. The factors involved in the pathophysiology of bladder hernias include bladder outlet obstruction (e.g. benign prostatic hyperplasia, or strictures of the bladder neck or urethra), obesity and decreased bladder tone and weakness of the pelvic musculature as occurs in the aging population [7, 9, 10]. In our case, infravesical obstruction in terms of prostate hyperplasia might be the reason for the herniated bladder.
Small bladder hernias are usually asymptomatic and are commonly diagnosed incidentally at the time of surgery [7]. Large inguinoscrotal bladder hernias classically present with two-stage micturition, where the first-stage occurs spontaneously, but the second-stage requires active manual compression of the hernia by the patient for  the residual urine to void. Non-specific urinary symptomatology, such as frequency, urgency, nocturia, dysuria and hematuria may occur secondary to bladder outlet obstruction or infection [7-10]. In the present case, inguinal tenderness during micturition, because of right groin soft tissue enlargement due to the hernia, was the patient’s complaint.
The diagnosis is made on clinical findings, with history, physical examination and radiological evaluation. Radiologic imaging is crucial in the diagnosis of bladder hernia, and the methods used are ultrasound, cystography, intravenous pyelography or CT scanning [7, 9]. CT and ultrasound, which are the current investigative modalities for scrotal cystoceles, can identify the anatomical deformity and any potential complications, such as bladder or bowel infarction. However, bladder hernia should be in the differential diagnosis if a fluid collection is identified in the groin on ultrasound. Other key diagnostic points include tissue in the hernia canal and scrotum with similar echogenicity to bladder tissue, alteration in the dimensions of the inguinal contents before and after voiding, and a visible bladder connection [9].
Imaging modalities, including CT, are rarely used to outline the details of inguinal hernias in practice, because surgical consultants generally proceed to surgery without imaging [11]. However, CT is the most important diagnostic modality, as it can identify the content of hernia (bowel/omentum) and rule out associated complications such as  strangulation. Imaging is not indicated in all cases of inguinal hernia, and the majority of bladder hernia are diagnosed incidentally during imaging performed for other indications. Some authors recommend that if there is a strong clinical suspicion of a bladder hernia, imaging (cystography or CT) should be performed preoperatively to delineate the anatomy of the sac and its contents to help reduce the risk of serious injury during herniorraphy [9, 12]. Gadodia et al. and Ansari et al. remarked that CT is the preferred imaging modality with high spatial resolution, not only to detect herniation, but also to show associated pathologies such as the content of the hernia (bowel, omentum), strangulation, and hydronephrosis [13, 14]. Our case gave us an idea about the evaluation of the herniated bladder with three-dimensional CT, which provides a less invasive method compared with cystography for assessing the relationship of the hernial sac with the bladder. In addition, three-dimensional CT can help preoperative surgical management and decrease the peroperative complication risk.
The standard treatment of inguinoscrotal bladder hernia is either reduction or resection of the herniated bladder followed by surgical repair. Bladder resection is recommended only for cases with bladder necrosis, a hernia neck of less than 0.5 cm in diameter, or a bladder diverticulum or tumor in the herniated bladder [8]. In our case, on three-dimensional CT, the  hernia neck was measured 2.8 cm in diameter and the reduction of bladder hernia was effortless without requiring bladder resection.

 

Conclusion
In conclusion, although conventional CT and cystography offer the routine imaging approach to locating a herniated bladder; three-dimensional CT is the best imaging technique and is also less invasive than cystography. In the near future, three-dimensional CT might replace all of these imaging modalities in order to demonstrate bladder herniation.

 

References
[1] Madden JL, Hakim S, Agorogiannis AB. The anatomy and repair of inguinal hernias. Surg Clin North Am. 1971 Dec: 51:1269-92
[2] Oruc MT, Akbulut Z, Ozozan O, Coskun F. Urological findings in inguinal hernias: a case report and review of the literature. Hernia. 2004 Feb: 8:76-9
[3] Bisharat M, O’Donnell ME, Thompson T, et al. Complications of inguinoscrotal bladder hernias: a case series. Hernia. 2009 Feb: 13:81-4
[4] Gilbert AI. Sutureless repair of inguinal hernia. Am J Surg. 1992 Mar: 163:331-5
[5] Kraft KH, Sweeney S, Fink AS, Ritenour CW, Issa MM. Inguinoscrotal bladder hernias: report of a series and review of the literature. Can Urol Assoc J. 2008 Dec: 2:619-23
[6] Wagner AA, Arcand P, Bamberger MH. Acute renal failure resulting from huge inguinal bladder hernia. Urology. 2004 Jul: 64:156-7
[7] Gomella LG, Spires SM, Burton JM, Ram MD, Flanigan RC. The surgical implications of herniation of the urinary bladder. Arch Surg. 1985 Aug: 120:964-7
[8] Thompson JE, Jr., Taylor JB, Nazarian N, Bennion RS. Massive inguinal scrotal bladder hernias: a review of the literature with 2 new cases. J Urol. 1986 Dec: 136:1299-301
[9] Casas JD, Mariscal A, Barluenga E. Scrotal cystocele: US and CT findings in two cases. Comput Med Imaging Graph. 1998 Jan-Feb: 22:53-6
[10] Karaman ZC, Saray A, Dorak C, Tamac NI. Ultrasonographic diagnosis of massive bladder hernia. J Clin Ultrasound. 1993 Oct: 21:534-6
[11] Schaeffer EM, Bhayani SB. Inguinal bladder hernia. Urology. 2003 Nov: 62:940
[12] Andac N, Baltacioglu F, Tuney D, Cimsit NC, Ekinci G, Biren T. Inguinoscrotal bladder herniation: is CT a useful tool in diagnosis? Clin Imaging. 2002 Sep-Oct: 26:347-8
[13] Ansari K, Keramati MR, Kalantari KR, Jafari M, Godazandeh G, Pakzad M. Gross hematuria as the presentation of an inguinoscrotal hernia: a case report. J Med Case Reports. 2011 Dec 4: 5:561
[14] Gadodia A, Sharma R, Parshad R. Bladder hernia: Multidetector computed tomography findings. Indian J Urol. 2011 Jul: 27:413-4

Date added to bjui.org: 24/03/2012


DOI: 10.1002/BJUIw-2011-143-web

Crossed fused ectopia with multicystic renal dysplasia

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We present a rare case in an infant who was found to have a congenital renal anomaly on routine second trimester antenatal ultrasound. 

Authors: Gupta, Pankaj; Goel, Sandeep; Sharma, Sanjay All India Institute of Medical Sciences (AIIMS), New Delhi, India

Corresponding Author: Sanjay Sharma, Deptartment of Radiology, All India Institute of Medical Sciences (AIIMS), New Delhi, India.  Email: [email protected]

 

Abstract
Crossed renal ectopia is the second most common fusion anomaly of the kidney after horse-shoe kidney. In 90% of patients, the crossed kidney fuses with the normal kidney. Multicystic dysplasia of the crossed (ectopic) kidney is relatively rare. Only a few isolated cases of crossed fused ectopia with multicystic dysplastic ectopic kidney have been reported. Though this is an unusual combination of rare renal anomalies, the radiologic features are characteristic enough to allow accurate preoperative diagnosis.  The entity can be suspected on antenatal ultrasonography (USG) and confirmed by postnatal USG and magnetic resonance urography (MRU). MRU is particularly useful to detect associated urinary tract abnormalities. We present one such rare case in an infant who was found to have a congenital renal anomaly on routine second trimester antenatal ultrasound.

 

Introduction
Renal ectopia is a common congenital malformation. Crossed ectopia occurs when a kidney lies on the contralateral side. It is second only to horse-shoe kidney as the most common congenital malformation [1],  and has an incidence of 1 in 7000 autopsies [2]. Most crossed ectopic kidneys fuse with the ipsilateral kidney resulting in crossed fused ectopia. Multicystic dysplastic kidney is a common cause of abdominal mass in infancy [3]. Histologically such kidneys show primitive metanephric structures [4]. Association of crossed fused ectopia with multicystic renal dysplasia is rare and presents an interesting appearance on imaging. We report one such case of multicystic dysplasia in an ectopic left kidney.

 

Case report
A five-month-old asymptomatic male infant presented for follow up of an abnormality that was recorded on antenatal ultrasound performed at 20 weeks of gestation. The antenatal ultrasound had shown the absence of a kidney in the left renal fossa with an abnormal multicystic structure related to the lower pole of the right kidney, which was structurally normal. The amniotic fluid volume and remainder of the ultrasound scan were reported as normal. The postnatal ultrasound scan showed a similar abnormality (figure 1a and b).

 

Figure 1.  Transverse ultrasound image through the right flank shows a cystic mass (figure 1a) in relation to the right kidney (figure 1b). The cysts are of variable sizes and are non-communicating. The left kidney was not found in the left flank.

 

Figure 1a. 

 

Figure 1b.

 

A presumptive diagnosis of crossed renal ectopia with dysplastic ectopic kidney was made. Blood urea and serum creatinine were normal. A DMSA scan revealed two areas of renal tissue on the right side. No renal tissue was found elsewhere in the abdomen or pelvis. A renal dynamic scan revealed mild right hydronephrosis with a non-functioning ectopic kidney. A micturating cystourethrogram (MCU) was performed to rule out lower urinary tract abnormalities. It revealed a small incidental diverticulum near the left vesicoureteric junction. No vesicoureteric reflux was noted and the urinary stream was normal. Magnetic resonance urography (MRU) was done to depict the anatomy of the entire urinary tract. MRU confirmed absence of the left kidney with a multicystic mass associated with the lower pole of the right kidney which was normal in position, shape and size with minimal hydronephrosis of the orthotopic right kidney. The cysts were non-communicating (figure 2).

 

Figure 2. Axial T2W images and coronal balanced true fast field echo (BTFE; Philips, Achieva 3T) shows a cystic mass composed of variable sized cysts which are non-communicating, related to the lower pole of the right kidney. Also noted is the absence of left kidney.
Figure 2a. 

 

 

Figure 2b.

 

The ureters could not be visualised. A diagnosis of crossed fused multicystic dysplastic ectopic left kidney was made. The infant is well, and continues to be followed up.

 

Discussion
Horseshoe kidney is the most common congenital renal anomaly. and crossed renal ectopia is the second most common, with a reported incidence of 1 in 7000 autopsies. It is reported to be more common in males and in most cases; the left kidney is crossed to the right [5]. The crossed kidney has an aberrant vascular supply [5], which can arise from either side of the lower abdominal aorta or from the common or external iliac arteries. During embryogenesis, normal renal development and position relies on interaction between the ureteric buds and nephrogenic cords. Crossed renal ectopia results when there is fusion of both the ureteric buds with only one nephrogenic cord [6].
Multicystic renal dysplasia is defined as a disturbed differentiation of nephrogenic tissue with the persistence of structures inappropriate to the age of the patient [7]. Multicystic renal dysplasia is characterised by the presence of cysts of varying sizes, pelvicalyceal and ureteric atresia and a non-functioning kidney.
Few cases of multicysticystic dysplasia of a crossed kidney (MCKD) have been previously reported [8-11]. Embryogenesis of this entity involves early in utero fusion of the two kidneys and variation in axial deviation of the fused renal elements. MCDK is believed to occur due to early ureteric obstruction caused by renal fusion. The underlying mechanism of ureteric obstruction may include pressure by the orthotopic kidney, abnormal vasculature, ureteric ectopia or ectopic ureterocele [12].
This rare and interesting entity should be considered in an infant who presents with a flank swelling caused by a multicystic mass caudal to the orthotopic kidney which may show variable degrees of hydronephrosis with absence of the contralateral kidney. Nussbaum et al [13] described the classical findings of multicystic dysplasia in crossed renal ectopy: a variable sized multicystic mass located adjacent to the lower pole of a hydronephrotic malrotated orthotopic kidney; ureteric displacement with or without dilatation; absence of the contralateral kidney and its renal artery. Hydronephrosis of an orthotopic kidney is frequently associated with this malformation and the most common cause for this is ureteric narrowing at the pelviureteric  junction. It occurs secondary to pressure caused by the caudal dysplastic kidney and the resultant ischaemia. The course of the ureter is altered; it may lie anterior to the mass, behind it or it may be deviated laterally.
Most of the features of MCDK are amenable to ultrasound visualisation, so a preoperative diagnosis can be made confidently based on ultrasound features. The characteristic ultrasound appearance of MCDK is the presence of multiple randomly distributed non-communicating cysts of variable sizes with the absence of peripheral parenchyma as well as an echogenic sinus complex. However, severe hydronephrosis can mimic the uncommon hydronephrotic form of MCDK [14]. With the advent of MRU, similar features may be shown exquisitely and with a more complete evaluation of both upper and lower urinary tract. The degree of hydronephrosis, presence of a ureter, and its course, are all useful information provided by MRU, without exposing infants to radiation from intravenous urography (IVU) or computed tomographic urography (CTU). A renogram is required to assess the function of the crossed kidney and a micturating cystourethrogram is indicated to identify abnormalities of the lower urinary tract.
Most multicystic kidneys undergo involution over the first few years of life and may disappear altogether [11, 15]. A number of studies have shown that MCDK can be reliably followed by ultrasonography. The majority of studies that followed children with MCDK with serial ultrasonography have demonstrated a high percentage of regression of the dysplastic kidney. This has led to a change in the management of multicystic kidneys [11, 15]. A low incidence of complications and a high incidence of spontaneous regression support conservative management in most cases (as was decided in our patient). Nephrectomy is reserved for masses that do not regress.
In conclusion, MCDK in an ectopic kidney should be suspected when antenatal USG shows a multicystic mass associated with a kidney and the absence of a contralateral kidney. The diagnosis can be made accurately with ultrasonography in most cases.. Mild and asymptomatic cases are followed conservatively with serial USG and those with severe disease are managed surgically.

 

References
 
1. Mouriquand P. Renal Fusions and Ectopia. In: JA O’Neill, MI Rowe, JL Grosfeld, EW Fonkalsrud, AG Coran, eds. 5th Edition. Pediatric Surgery. Mosby: St Louis, 1998:1583-1588.
2. Hertz M, Rubinstein ZJ, Shahin M, Malzer M. Crossed renal ectopia: clinical and radiological findings in 22 cases. Clin radiol 1977; 28:339-344
3. Mohammad M. Association of multicystic dysplasia and crossed non fused renal ectopia: a case report. SJKDT 1997: 8(21):148-151.
4. Bernstein I. Developmental abnormalities of renal parenchyma, renal hypoplasia and dysplasia. Pathobiol Annu 1968; 3: 213-215.
5. Abeshouse BS, Bhisitkul I. Crossed renal ectopia with or without fusion. Urol Int 1959; 9: 63-91.
6. Cook WA and Stephens FD: Fused kidneys: morphologic study and theory of embryogenesis. Birth Defects Orig Artic Ser 1977; 13: 327.
7. Kissane JM. Congenital malformations in pathology of the kidney. Edited by Hepinstall RH. Boston, Little Brown. 1956. Pp. 63-117.
8. Evan WP, Sumner TE, Lorentz WB Jr, Rosnick MI. Association of crossed fused renal ectopia and multicystic kidney.  J Urol 1979; 122: 821-22
9. Herczeg T, Rutkai P, Deak J. Metanephrogenic cystic remnant of crossed dystopic kidney. J Urol 1956; 76: 488-498
10. Rosenberg HK, Synder HM III, Duckett I. Abdominal mass in a new-born: multicystic dysplasia of crossed renal ectopia- ultrasonic demonstration. J Urol 1984; 131: 1160-1161
11. Sahu SS, Maheshwari UM, Borkar DB, Sachdeva HS. Unilateral multicystic dysplasia in an ectopic kidney. Indian J Pathol Microbiol 2011;54:437-8
12. Daniel WW, Datnow B.  Crossed fused ectopia with renal dysplasia. Am J Roentgenol 1977;128: 845-846
13. Nussbaum AR, Hartman DS, Whitley N, et al. Multicystic dysplasia and crossed renal ectopia. AJR Am J Roentgenol 1987;149:407-410.
14. Sanders RC, Hartman DS. The sonographic distinction between neonatal multicystic dysplastic kidney and hydronephrosis. Radiology 1984; 151: 621-625
15. Rottenberg GT, Gordon I, Bruyn R De. The natural history of multicystic  dysplastic  kidney in children. BJR 1997; 70: 347-350

Date added to bjui.org: 21/03/2012


DOI: 10.1002/BJUIw-2011-100-web

Isolated late bony recurrence in malignant teratoma

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We present a 65 year old man who presented with a three year history of right testicular swelling and weight loss. 

Authors: Deivasikamani Ramanujam Venkatachala1, Elaine MacDuff 2, Karen Smith 3 , Mike Jane3, Ed Kalkman1, Martin Russel1, Ashita Waterston1 ,Jeff White1.

1. The Beatson West of Scotland Cancer Centre, Glasgow.
2. Western Infirmary, Glasgow.
3. Glasgow Royal Infirmary, Glasgow.

Corresponding Author: Deivasikamani Ramanujam Venkatachala,  The Beatson West of Scotland Cancer Centre, Glasgow. E-mail: [email protected]

 

Introduction
 
Testicular germ cell tumours are the most common cancer of young men. They respond well to treatment, with anticipated cure rates of over 90%. Late recurrences two years after initial treatment are uncommon; and very late recurrences beyond five years of initial treatment are rare. However they are reported in Non Seminomatous Germ Cell Tumour (NSGCT).
Malignant teratoma is a type of NSGCT characterized by the presence of undifferentiated elements and the potential for distant metastasis. Bony involvement in malignant teratoma is rare, and isolated bone involvement is very rare.
Tumour markers are helpful in identifying early recurrence in malignant teratoma, emphasising the need for serial measurements and longer follow up. 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET) scanning plays an important role in identifying the site of recurrence in marker only recurrence in malignant teratoma.
Late recurrences in malignant teratoma arise from differentiated elements. They are best treated by surgery, aiming for complete resection to achieve cure .Management of iliac bone metastasis is complex and involves input from various specialities in a multidisciplinary team setting. Chemotherapy and radiotherapy help to palliate symptoms and improve quality of life.

 

Case Report
 
We present a 65 year old man who presented with a three year history of right testicular swelling and weight loss. Further investigations revealed malignant teratoma with multiple pulmonary nodules and no other metastases. His tumour markers were raised with a Human Chorionic Gonadotropin (HCG) of 1253 mIU/ml. He had an intermediate risk tumour in the International Germ Cell Cancer Collaborative Group (IGCCCG) prognostic classification.
He was treated with orchidectomy and four cycles of BEP (Bleomycin, Etoposide and Platinum) chemotherapy with a good response. He achieved remission with complete resolution of the pulmonary nodules and his tumour markers normalised. A follow up CT scan did not show any evidence of residual disease to warrant post chemotherapy surgery. The left iliac bone was slightly prominent, suggestive of Paget’s disease. He was asymptomatic and was followed up regularly in the Germ Cell Cinic.
Seven years later his alpha fetoprotein (AFP) level increased gradually, and remained between 8 and 37ku/l. (Figure 1).

 

Figure 1. Alpha fetoprotein (AFP) level increased gradually

 
He was clinically well without any symptoms. He had various scans during this period including a FDG- PET/CT scan which showed no evidence of recurrence. However, the left iliac bone appeared irregular with a trabecular pattern and was metabolically inactive. The bone changes were similar to the appearances on CT scan at the time of presentation. It was concluded that this was  either fibrous dysplasia or Paget’s Disease following discussions in the multi disciplinary meeting.
Six months later his AFP increased to 100ku/l. Further imaging with FDG- PET/ CT scanning showed a metabolically active lesion in the tail of the pancreas as well as the left iliac bone. He was clinically well without any symptoms. He was discussed in the multi disciplinary meeting. The bone appearances in the iliac bone remained unchanged on the CT scan except being metabolically active, and he was asymptomatic. The abnormal activity in the iliac bone was again concluded as being secondary to either fibrous dysplasia or Paget’s Disease. A  pancreatic biopsy was planned to exclude recurrence in the pancreas.
Meanwhile the patient developed hip pain and his AFP progressively increased to 6,000ku/l. Further FDG -PET/CT scan showed persistent activity in the iliac bone (Figure 2 &3) along with a soft tissue mass, and the pancreatic lesion disappeared.

 

Figure 2. FDG -PET/CT scan

 

Figure 3. 

 

Subsequently he had biopsy of the left iliac bone which confirmed metastatic teratoma of yolk sac tumour with atypical glandular and papillary growth pattern. (Figure 4 &5)

 

Figure 4. 

 

Figure 5. 

 

At this stage he was considered for radical surgery. As the teratoma was diffusely infiltrating the iliac bone and the sacrum, it was thought that radical surgery even with a hind quarter amputation would not achieve clear margins and would result in considerable disability. He was treated with radical radiotherapy and received 50 Gy in 25 fractions to the iliac bone and pelvis. He had good clinical response and his AFP reduced to 2162 ku/l.
However in the next six months his hip pain worsened and the AFP progressively increased to 26100 ku/l. At this stage further treatment options were discussed and he had less radical surgical curettage and cementoplasty of the iliac bone. Following this he had a good clinical response, his pain improved significantly and his AFP dropped to 6100 ku/l.
Within three months his hip pain worsened and his AFP progressively increased to 11,100 ku/l.  He was treated with six courses of palliative Gemcitabine and Oxaliplatin chemotherapy. He had a good clinical and metabolic response and his AFP dropped to 26 ku/l.
Within two months of completing chemotherapy his AFP progressively increased to 650 ku/l. He had two courses of Cisplatin and Etoposide with some response but this was discontinued due to myelotoxicity.
He was considered for experimental treatment but was not eligible for any current studies. He currently remains well and active with symptomatic treatment alone and enjoys a good quality of life.

 

Discussion
Patients with germ cell tumours respond well to treatment. Patients who remain in remission for more than two years following initial treatment are considered to be cured. [1] Most recurrences occur within two years of treatment; late recurrences beyond two years of initial therapy are uncommon.
Very late recurrences beyond five years are rare and occur in Non Seminomatous Germ Cell Tumour; with a 1% annual risk of recurrence between five and ten years.[4] The commonest sites of late relapse are abdominal (in the paraaortic, and retroperitoneal regions), and occur in about 50 % of the patients. [2, 4] Positive tumour markers at presentation and the presence of differentiated elements in the tumour after chemotherapy are important predictive factors of late recurrence. [4]
Clinically detectable bone metastasis occurs in 3% of patients with metastatic germ cell tumour at presentation [6]. The incidence of late bone metastasis varies between 3- and 9% [6, 7] and isolated late bone recurrence is very rare, seen in 1% of the patients. [7] The bones of the trunk, especially the lumbar spine are commonly involved as described in historical autopsy series [8, 9] and pelvic bone involvement is very rare.
Long term follow up beyond five years is vital in patients with NSGCT presenting with positive tumour markers and advanced stage at diagnosis. Follow up usually involves clinical assessment, chest radiographs and measurement of tumour markers.
Serial measurement of tumour markers is useful in diagnosing late recurrences as they are elevated in greater than 50% of the recurrences. [4] Elevated tumour markers are one of the earliest and sole indicators of recurrence as seen in our patient. [4] FDG-PET /CT scan is valuable in localising the site of recurrence in patients with marker only relapse. [10]
Most of the late recurrences in NSGCT after initial chemotherapy are believed to arise from residual differentiated teratoma elements. Late recurrences with yolk sac differentiation reveal atypical glandular and papillary growth pattern. [11] They have a different natural history characterized by slow growth, secretion of AFP, chemo resistance and poor prognosis. [3, 11]
Surgery plays the most important role in the management of late recurrence. [2, 4] Complete resection of viable tumour increases the chance of cure. However with incomplete resection the recurrence rate is higher. Chemotherapy and radiotherapy helps in palliating symptoms and thereby improves quality of life.
Conclusion
Isolated bone metastasis in Non Seminomatous Germ Cell Tumour is very rare. Management of iliac bone disease is complex and should involve a multi disciplinary team approach.
Prolonged follow up beyond five years with measurement of tumour markers is important, especially for those presenting with positive tumour markers and advanced stage at presentation.
FDG-PET/ CT scan plays a vital role in localising relapse site in marker only recurrences.
Late recurrences have a different natural history. Surgery plays the most important role in the management of late recurrence.
Chemotherapy and radiotherapy help in palliating symptoms and thereby improve quality of life.

 

References
1, Einhom LH-: Testicular cancer as a model for a curable neoplasm: The Richard and Hilda Rosenthal Foundation Award Lecture. Cancer Res. 1981; 41:3275-3280.
2, Baniel J, Foster RS, Gonin R ,Messemer JE,Donohue JP, Einhom LH. Late relapse of testicular cancer. J Clin Oncol.1995; 13(5): 1170–1176.
3, Gerl A, Clemm C, Schmeller N, Hentrich M, Lamerz R, Wilmanns W. Late relapse of germ cell tumors after cisplatin-based chemotherapy. Ann Oncol. 1997; 8:41–47.
4, Shahidi M, Norman AR, Dearnaley DP, Nicholls J, Horwich A, Huddart RA. Late Recurrence in 1263 Men with Testicular Germ Cell Tumors. Cancer. 2002; 95:520-530.
5, Oldenburg J, Alfsen GC, Waehre H, Fossa SD. Late recurrences of germ cell malignancies: a population-based experience of over three decades. Br J Cancer.2006; 94:820-827.
6, Hitchins RN ,Philip PA, Wignall B, et al. Bone disease in testicular and extragonadal germ cell tumours. Br. J. Cancer.1988; 58:793-796.
7, Flechon A, Culine S, Theodore C, Droz JP. Pattern of Relapse after First Line Treatment of Advanced Stage Germ-Cell Tumors. European Urology. 2005; 48: 957–964.
8, Dixon FJ and Moore RA. Testicular tumours – A clinicopathologic study. Cancer. 1953; 6(3): 427- 454.
9, Mostofi FK .Testicular tumours – Epidemiologic, etiologic, and pathologic features. Cancer. 1973; 32(5), 1186 – 1201.
10, Hain SF, O’Doherty MJ, Timothy AR, Leslie MD, Harper PG and Huddart RA. Br J Cancer.2000; 83(7): 863-869.
11, Michael H, Lucia J, Foster RS, and Ulbright TM, American Journal of Surgical Pathology.2000; 24(2): 257–273.

Date added to bjui.org: 15/03/2012 


DOI: 10.1002/BJUIw-2011-135-web

Primary prostatic intravascular large B cell lymphoma

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Primary prostatic intravascular large B cell lymphoma is extremely rare; to date only six cases have been described.

Authors: 1.Wen-Chih Huang, M.D. Visiting Staff, Department of Anatomic Pathology, Far Eastern Memorial Hospital, Taipei, Taiwan

2.Huang-Chun Lien, M.D. Visiting Staff, Department of pathology, National Taiwan University Hospital, Taipei, Taiwan
3.Wei-Chou Lin M.D. Visiting Staff, Department of pathology, National Taiwan University Hospital, Taipei, Taiwan
4.Yi-Shuan Lee M.D. Visiting Staff, Department of pathology, Chia-Yi Christian Hospital, Chia Yi, Taiwan

Corresponding Author: Huang-Chun Lien, M.D.Department of pathology, National Taiwan University Hospital, Taipei, TaiwanNo.7, Chung Shan S. Rd. Taipei, TaiwanPhone: +886-912013231Fax: +886-2-77282060E-mail: [email protected]

 

Introduction
Prostatic lymphomas are rare, and primary prostatic intravascular large B-cell lymphoma is extremely rare. Here we present a male patient with obstructive voiding symptoms and the clinical impression was of benign prostate hyperplasia. Histopathologic features revealed large neoplastic lymphoid cells within the lumina of small to medium-sized vessels. These cells were positive for B cell immunophenotype, indicating an intravascular large B cell lymphoma. Pathologists and clinicians should be aware of the possibility of malignant lymphoma in patients with obstructive uropathy.

 

Case report
An 88-year-old male presented with a many year history of obstructive voiding symptoms. His past medical history included hypertension for which he was regularly followed up. Digital rectal examination revealed enlargement of the prostate and his prostate specific antigen level was 7.31ng/ml. A full blood count showed anemia, with a Hemoglobin level of 6.8g/dl. The patient’s lactate dehydrogenase (LDH) level was 388 IU/L (normal range: 135-225 IU/L). His other laboratory data was within normal limits. Transurethral resection of the patient’s prostate was performed.
Histopathology findings were of a background of nodular hyperplasia, with multiple foci of tumor lodged in the capillaries and post-capillary venules demonstrated in all sections of the specimen (figure 1).

 

Figure 1 Large neoplastic lymphoid cells within the lumina of small to medium-sized vessels were present. (H&E, original magnification ×200)

 

The tumor cells were large, with prominent nucleoli and frequent mitotic figures (figure 2).

 

Figure 2 The tumor cells were large-sized with nucleoli and mitotic figures. (H&E, original magnification ×200)

 

Immunohistochemically, the atypical cells were positive for CD20 (figure 3).

 

Figure 3 These lymphoma cells expressed CD20. (immunohistochemical staining, original magnification ×100)

 

CD31 immunostaining highlighted the endothelial cells of the vessels, confirming the presence of neoplastic cells within the vessels (figure 4).

 

Figure 4 CD31 immunostain highlighted the endothelial cells of the vessels, containing lymphoma cells in the vascular lumen. (immunohistochemical staining, original magnification ×200)

 

Ki-67 demonstrated high proliferative activity in the neoplastic cells (figure 5).

 

Figure 5 Ki-67 demonstrated high proliferative index in the neoplastic cells. ((immunohistochemical staining, original magnification ×200)

 

Intravascular large B cell lymphoma was diagnosed. Further CT scanning revealed the appearance of an enlarged prostate post transurethral resection (figure 6).

 

Figure 6 Computed tomography revealed an enlarged prostate post transurethral resection.

 

No regional lymphadenopathy was observed. Despite chemotherapy and supportive treatment, the patient died two months after diagnosis.

 

Discussion
The patient presented with elevated serum lactate dehydrogenase and anemia. All sections of the prostate revealed multiple foci of tumor cells lodged in the vascular lumina. Ki-67 demonstrated high proliferative activity in the neoplastic cells. No extravascular tumor mass or lymphadenopathy was found in computed tomography. According to these features, primary prostatic intravascular large B-cell lymphoma was diagnosed.
Primary prostatic lymphoma is extremely uncommon, and fewer than 200 cases have been reported [1]. Secondary prostate involvement by systemic lymphoma is much more common. Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma in the World Health Organization classification [2]. In the past, the majority of cases were diagnosed at autopsy. A wide range of different organ involvement has been described, including the skin, central nervous system, adrenal glands, lungs, gastrointestinal system, kidneys and genitourinary tract. [3, 4]. The clinical presentation is varied and often includes symptoms related to the organ involved. In Western countries, the clinical manifestations are usually neurologic and dermatologic symptoms [5]. In Asia, neurological symptoms and cutaneous lesions are less common than in Western countries [6]. A few cases of IVLBCL associated with hemophagocytic syndrome have been described in Asia [7].
Primary prostatic IVLBCL is extremely rare; to date only six cases have been described [8, 9]. These patients with prostatic lymphoma usually exhibit obstructive and irritative voiding symptoms, difficult to distinguish from benign prostatic hyperplasia or carcinoma of the prostate. Accurate and timely diagnosis is still a problematic issue. Microscopically, large neoplastic lymphoid cells within the lumina of small to medium-sized vessels are present. These cells express B cell phenotype. The differential diagnosis of IVLBCL may include inflammatory or immune reactions with activated lymphocytes within the post-capillary venules. Combining the clinical symptoms, evidence of organ dysfunction, laboratory abnormalities, histopathology and immunohistochemical studies can establish the diagnosis.
Cases of IVLBCL are considered disseminated due to presence of lymphoma cells in the vessels and are therefore treated with systemic chemotherapy. A combination of doxorubicin, cyclophosphamide, vincristine, prednisone and anti-CD20 antibody rituximab (R-CHOP) is the most commonly employed regimen [10, 11]. Patients with an early diagnosis and aggressive chemotherapy may achieve a higher response rate and long-term survival [12].

 

Conclusion
Primary prostatic intravascular large B-cell lymphoma is extremely uncommon; to date only six cases have been described. Patients with prostatic lymphoma usually exhibit obstructive and irritative voiding symptoms, difficult to distinguish from benign prostatic hyperplasia or carcinoma of the prostate. Pathologists and urologists should be aware of the possibility of malignant lymphoma in patients with obstructive uropathy.

 

Reference
 
1. Iczkowski KA, Lopez-Beltran A, Sakr WA: Hematolymphoid tumors of prostate. In: World Health Organization classification of tumors. Pathology and genetics of tumors of the urinary system and male genital organs, 3rd ed. Edited by Eble JN, Sauter G, Epstein JI, et al. Lyon, France: IARC Press, 2004: p212.
2. Nakamura S, Ponzoni M, Campo E: Intravascular large B-cell lymphoma. In: World Health Organization classification of tumors of haematopoietic and lymphoid tissues, 4th ed. Edited by Swerdlow SH, Campo E, Harris NL, et al. Lyon, France: IARC Press, 2008: p252-253.
3. Dermier T, Dail DH, Aboulafia DM. Four varied cases of intravascular lymphomatosis and literature review. Cancer 1994; 73: 1738–1745.
4. Murase T, Yamaguchi M, Suzuki R, et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood 2007; 109: 478-485.
5. Chapin JE, Davis LE, Kornfeld M, Mandler RN. Neurologic manifestations of intravascular lymphomatosis. Acta Neurol Scand 1995; 91: 494-499.
6. Shimada K, Murase T, Matsue K, et al. Central nervous system involvement in intravascular large B-cell lymphoma: a retrospective analysis of 109 patients. Cancer Sci 2010; 101: 1480-1486.
7. Murase T, Nakamura S, Kawauchi K, et al. An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol 2000; 111: 826–834.
8. Csomor J, Kaszás I, Kollár B, et al. Prolonged survival using anti-CD20 combined chemotherapy in primary prostatic intravascular large B-cell lymphoma. Pathol Oncol Res. 2008;14: 281-284.
9. Xu M, Yang Q, Li M, Geng W, Huang W, Chen Y. Prostate involvement by intravascular large B-cell lymphoma: a case report with literature review. Int J Surg Pathol. 2011; 19: 544-547.
10. Ferreri AJ, Dognini GP, Govi S, et al. Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma? J Clin Oncol 2008; 26: 5134-5136.
11. Shimada K, Matsue K, Yamamoto K, et al. Retrospective analysis of intravascular large B-cell lymphoma treated with rituximab-containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol 2008; 26: 3189-3195.
12. Ponzoni M, Ferreri AJ, Campo E, et al. Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol 2007; 25: 3168-3173.

Date added to bjui.org: 10/03/2012 

DOI: 10.1002/BJUIw-2011-102-web

Management of chylous lymphorrhea after retroperitoneal surgery

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We describe the management of a patient who suffered from massive chylous lymphorrhoea after retroperitoneal surgery for a cystic lymphangioma.

Authors: Bekkali, Sami; Bialek, David; Entezari, Cedric; Entezari, Kim

Corresponding Author: Sami Bekkali, Military Hospital,  Department of urology, Rabat, Morocco. Email: [email protected]

 

Abstract
The surgical management of testicular or renal cancer with retroperitoneal lymph node dissection (RPLND) has potential risks and complications. One such complication is chylous lymphorrhoea.It represents a difficult management problem due to serious mechanical, nutritional and immunological consequences of the constant loss of protein and lymphocytes in the ascitic fluid.
As we move to more frequent lymphadenectomy for renal cell carcinoma and because of the lack of data concerning the management of  persistant and massive chylous lymphorrhoea, it is interesting for the urologist to be aware of different conservative treatments.
We describe the management of a patient who suffered from massive chylous lymphorrhoea after retroperitoneal surgery for a cystic lymphangioma.
Introduction
 
Lymph node dissection represents the most accurate and reliable staging procedure for lymph node invasion in urological cancers and remains the gold standard for nodal assessment.
Chylous lymhorrhoea is an infrequent complication of retroperitoneal surgery that usually develops as a result of operative trauma caused by the interruption of major lymphatic channels[1]. In a retrospective study by Press et al[2], it occurred with a frequency of 1 out of 20,664 hospital admissions. In a study by Baniel et al, its incidence was of 2 % [3]. Increased chyle production and obstruction of the abdominal lymphatic drainage may further contribute to the pathogenesis[4].
Although it remains a rare complication of  RPLND, its management becomes challenging for the urologist when the lymphorrhoea is massive and persistent.
We report a case of refractory chylous lymphorrhoea following retroperitoneal surgery for a cystic lymphangioma that did not respond to conventional conservative measures and resolved rapidly after administration of somatostatin.

 

Case report
 
A 50 year-old woman presented with left abdominal pain. An abdominal CT scan revealed a 13×9×6cm retroperitoneal mass for which she underwent laparotomy. Final pathological examination revealed a cystic lymhangioma. She was discharged, well,  on the sixth post operative day.
Three weeks after the operation, she presented complaining of abdominal pain. Evaluation with abdominal tomography (figure 1) revealed a large left retroperitoneal collection 21×10×11 cm in size. A chylous lymphorrhoea was suspected and the patient was hospitalized for conservative management.

 

Figure 1. Abdominal tomography revealed a large left retroperitoneal collection 21×10×11 cm in size. 

CT guided drainage of the collection was performed and yielded 4200 ml of milky fluid. Laboratory analysis revealed a triglyceride level of 858 mg/dl, cholesterol of 69 mg/dl and an albumin level of 2 g/dl which is biochemically compatible with chyle.
Oral intake was discontinued and total parenteral nutrition (TPN) was commenced. The drainage liquid became clear within 36 hours but continued to yield between 1000 and 1500 ml per 24 hours. Ten days after hospitalization, continuous intravenous administration of  somatostatin 6mg per 24 hours was started. The drainage liquid started decreasing significantly the following day and reduced to an insignificant amount within a few days. After one week, the somatostatin dose was decreased to 3mg per day for two days then stopped, the drain was removed, parenteral nutrition was discontinued and oral intake allowed with a restricted fat content.
A CT scan was performed  once the somatostatin therapy stopped and showed a significant regression of the collection (figure 2).

 

Figure 2. CT scan after somatostatin therapy

 

The patient was discharged and returned a month later for a further CT (figure 3) which demonstrated that the collection had almost completely resolved.

 

Figure 3. Futher CT scan

 

Discussion
 
Somatostatin is a naturally occurring peptide hormone, present in the central nervous system, the gastrointestinal tract and the pancreas[5]. The exact mechanisms involved in the “ drying effect “ of somatostatin on lymhorrhoea are not completely understood. Nevertheless, somatostatin was previously shown to reduce intestinal absorption of fat and attenuate lymph flow in major lymphatic channels[6]. In addition, it also reduces gastric, pancreatic and intestinal secretions and decreases splanchnic blood flow which may further contribute to the decreased lymph production[7].
The initial use of somatostatin to treat post operative lymphorrhoea was reported by  Ulibarri et al[8]. Subsequently, others also achieved satisfactory results in the treatment of lymphorrhoea using somatostatin[9,10]. Typically, the response to continuous intravenous administration of somatostatin is characterized by a drastic decrease in the output, following a couple of days’ treatment. Rapid resolution of postoperative lymphorrhoea was also reported with subcutaneous administration of octreotide[11].
In some cases chylous lymphorrhoea may take a protracted and complicated course in spite of maximal conservative measures, necessitating  invasive measures such as peritoneovenous shunt or direct surgical lymphostasis.

 

Conclusion
 
The treatment of postoperative chylous lymphorrhoea is primarily conservative, intending to reduce the lymph flow in the disrupted retroperitoneal lymphatics. A trial of somatostatin therapy should be included early in the course of treatment before any invasive measures are undertaken.

 

References
1. Nurettin Boran, Aylin Pelin Cil, Gokhan Tulunay, Nejat Ozgul, M. Faruk Kose. Gynecologic Oncology, Volume 93, Issue 3, June 2004, Pages 711-714
 2.O.W. Press, N.O. Press and S.D. Kaufman, Evaluation and management of chylous ascites. Ann Intern Med,  96  (1982), p. 358.
3.J. Baniel, R.S. Foster and R.G. Rowland, et al complication of primary lymph node dissection. J Urol,  152 (1994), pp. 424–427
4. Ilan Leibovitch, Yoram Mor, Jacob Golomb, Jacob Ramon. European Urology, Volume 41, Issue 2, February 2002, Pages 220-222
 5.N.J. Demos, J. Kozel and J.E. Scerbo.  Somatostatin in the treatment of chylothorax. Chest 119 (2001), pp. 964–966
6. J.M. Collard, P.F. Laterre, F. Boemer, M. Reynaert and R. Ponlot. Conservative treatment of postsurgical lymphatic leaks with Somatostatin-14. Chest 117 (2000), pp. 902–905
7. P.C. Rimensberger, B. Muller-Schenker, A. Kalangos and M. Beghetti. Treatment of a persistent postoperative chylothorax with Somatostatin. Ann. Thorac. Surg. 66 (1998), pp. 253–254.
8. .I. Ulibarri, Y. Sanz, C. Fuentes, A. Mancha, M. Aramendia and S. Sanchez. Reduction of lymphorrhagia from ruptured thoracic duct by Somatostatin (letter). Lancet 336 (1990), p. 258.
9. K. Al-Sebeih, N. Sadeghi and S. Al-Dhahri. Bilateral chylothorax following neck dissection: a new method of treatment. Ann. Otol. Rhinol. Laryngol. 110 (2001), pp. 381–384.
10. R.F. Kelly and S.J. Shumway. Conservative management of postoperative chylothorax using Somatostatin. Ann. Thorac. Surg. 69 (2000), pp. 1944–1945.
11. M. Ferrandiere, E. Hazouard, V. Guicheteau, A. Gouchet, M. Bensenouci, C. Lamotte and C. Mercier. Chylous ascites following radical nephrectomy: efficiency of octreotide as treatment of a ruptured thoracic duct. Intensive Care Med. 26 (2000), pp. 484–485

 

Date added to bjui.org: 02/03/2012

DOI: 10.1002/BJUIw-2011-094-web

 

Micropapillary Urothelial Carcinoma (MUC) of the Upper Urinary Tract: An Underreported but Aggressive Variant of Urothelial Carcinoma

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We report 3 patients with MUC of the upper urinary tract who were evaluated and treated at our center between 2009 and 2011.

Authors: Xiao-hua Zhang1，Zhou-jun Shen1，Xiu-ling Wu2，Zhixian Yu3

  1. Department of urology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
  2. Department of pathology, The First Affiliated Hospital of Wenzhou Medical College, Wenzhou, Zhejiang, 325000, China
  3. Department of urology, The First Affiliated Hospital of Wenzhou Medical College, Wenzhou, Zhejiang, 325000, China

Corresponding Author: Zhixian  Yu,   Department of urology, The First Affiliated Hospital of Wenzhou Medical College, Wenzhou, Zhejiang, 325000, China. E-mail: [email protected]

Abstract 

We report 3 patients with MUC of the upper urinary tract who were evaluated and treated at our center between 2009 and 2011. A comprehensive Pubmed search of all case reports and literature related to MUC of the upper urinary tract was performed. In all cases, the tumors were located in the renal pelvis. All 3 patients were treated surgically. After a median follow-up of 12 months, median overall survival was 12 months. One patient presented with stage II, one with stage III and one with stage IV disease. The patient with stage II  disease is alive, with limited follow up showing no evidence of disease. The patient with stage III disease developed metastasis to the cervical lymph nodes 3 months later. Radiotherapy was performed, however she died 6 months after the initial diagnosis. The patient with stage IV disease, underwent systematic chemotherapy, but developed distant metastases (including port-site metastasis) and died 12 months after initial diagnosis. After a comprehensive search in PubMed/Medline, 50 cases have been reported. Thirty cases originated in the renal pelvis, and most patients died after surgery. MUC of the upper urinary tract often presents at an advanced stage with lymphovascular invasion and distant metastasis, and is probably an underreported variant of urothelial carcinoma associated with poor prognosis. The optimal treatment strategy is yet to be defined.

Introduction 

Micropapillary urothelial carcinoma (MUC) is an aggressive variant of urothelial carcinoma with high metastatic potential. Amin et al first reported MUC of the urinary bladder in 18 cases[1].This variant demonstrated a high tendency to invade lymphovascular spaces and to metastasize to lymph nodes and other organs. They also found MUC to be associated with advanced tumor stage and, therefore, a poor prognosis. Several other studies have confirmed these observations in MUC of the urinary bladder[2-4]. Although MUC has been well studied in the urinary bladder, there have been only a few studies of this tumor in the upper urinary tract, including the renal pelvis and ureter[5-13].The analysis of 3 consecutive cases of MUC of the upper urinary tract, may add to the current literature regarding this aggressive disease entity and may help with its future management.The literature with particular emphasis on the diagnostic and therapeutic approaches is also included and discussed.

Patients and methods

Clinical Case

After obtaining approval from the institutional review board, we retrospectively searched the files of all patients with urothelial carcinoma of the upper urinary tract (including the renal pelvis and ureter) treated at our center between 2009 and 2011. Patients were considered to have micropapillary disease if the pathology report revealed any micropapillary component (MC) in their tumor. Patient medical records were analyzed for demographic characteristics, clinical stage and outcome. Radiology, pathology and surgical reports were reviewed to determine the pathological staging at the time of nephro-ureterectomy using the 1997 TNM classification for genitourinary tumors [14]. Chemotherapy regimens, radiotherapy doses and surgical modality were also recorded. Overall survival was calculated from the date of diagnosis to the date of death or the date of last follow-up.

Search Strategy and Systematic Literature Review

We performed a comprehensive search in PubMed/Medline, up to September 2011, using the terms ‘micropapillary’, ‘renal pelvis’, ‘ureter’, ‘upper urinary tract’, ‘micropapillary urothelial carcinoma’. Articles were limited to the English language. The initial search was limited to articles from 1994 to 2011 due to micropapillary urothelial carcinoma being first reported in 1994. The inclusion criterion were original articles describing the micropapillary urothelial carcinoma of the upper urinary tract, including the renal pelvis and ureter. Exclusion criteria were articles published on the micropapilary urothelial carcinoma of urinary bladder. A total of 11 articles were included in the final analysis.

Results 

Clinical Results

Characteristics of patients at initial presentation

Between 2009 and 2011, 87 patients with urothelial carcinoma of the upper urinary tract were treated at our center. Three patients had micropapillary features. In all cases, the tumor was located in the renal pelvis. Median age at diagnosis was 67 years (59 to 75 years) and male/female ratio was 2/1. Two patients had previously smoked. The presenting symptom was macroscopic hematuria in two patients, and flank pain in one. Two of these patients had macrohematuria and were in a poor general condition, while one female patient had been treated with external beam radiotherapy for cervical lymph node metastases 5 years previously. All patients had grade 3 urothelial carcinoma and one had an associated in situ carcinoma. Lymphovascular invasion was present in all three cases (Fig.1-2.).

Figure 1. 

Figure 2. 

Metastases to regional lymph nodes were documented in two patients at the time of surgery, and to the ipsilateral adrenal gland in one patient. One patient presented with stage II, one with stage III and one with stage IV disease. The clinical characteristics of the patients are summarized in [Table 1].

Table 1. Summary of Clinical Characteristics

Case No. Age Sex Location Stage  Management Follow up
1 59 Male Renal pelvis T2 ONU* alive, no evidence ofdisease
2 67 Female Renal pelvis T3 LNU**+R^ 6 mo, died of metastasis tocervical lymph node
3 75 Male Renal pelvis T4 LNU+C# 12 mo,died of metastases tothe lung and liver.Port-stie metastasis was presented

*ONU,Open Nephro-Ureterectomy;**LNU,Laparoscopic Nephro-Ureterectomy; ^R,Radiotherapy; #C,Chemotherapy

Treatment and outcome 

One patient received open nephroureterectomy (ONU). He was alive, with limited (18months) follow up, and  showed no evidence of disease. One patient had only regional lymph node metastases, she underwent laparoscopic nephroureterectomy (LNU) and regional lymph node dissection. Her disease metastasised to her cervical lymph nodes 3 months later. Radiotherapy was performed, however she died 6 months later. One patient’s disease metastasised to the ipsilateral adrenal gland, he received LNU and excision of the affected adrenal gland. Although systemic chemotherapy was administered with gemcitabine 1000 mg/m 2 on days 1, 8 and 15,and cisplatin 70 mg/m2 on day 1 in a 28-day cycle, the disease still progressed and the patient died of metastases to his lungs and liver 12 months after surgery.

Metastatic sites

Metastases to regional lymph nodes were documented in 2 patients at the time of surgery; to the ipsilateral adrenal gland in one patient. Two out of the three patients developed distant metastases during follow up. The most frequent solid metastatic site was the lung followed by liver. One patient who underwent LNU developed port-site metastasis.

Survival data

At a median follow up of 12 months, 2 patients have died. One year survival was 66.7% and median survival was 12 months.

Systematic Review of Published Cases

After comprehensive search in PubMed/Medline, a total of 11 articles were included in the final analysis. We performed a bibliometric analysis finding a total of 50 cases of MUC of the upper urinary tract [Table 2]

Table 2. Clinical features of MUC of the upper urinary tract in literatures

Investigator Year No.cases Age*  M/F** Site(No.)# Stage(No.) Managment(No.) Follow up(No.)
Ribé 1996 1 68 1/0 P(1) T4(1) NU^+R^^+C^^^(1) 12mo,died of gastric metastases
Oh 2000 1 79 1/0 U(1) T4(1) NU+R(1) 20mo, died of gastric and peritoneal metastases
Vang 2000 1 79 0/1 U(1) T1(1)  laser ablation therapy(1) not available
Alvarado-Cabrero 2005 1 – – U(1) T3(1) – not available
Holmäng 2006 26 69 17/9 P(19)U(6)UP(1) T1(3)T2(1)T3(18)

T4(4)

 NU(14)UR^^^^(3)No¤(3)

NU+R(4)

NU+C(2)

 died of tumor(20)died of other diseas(6)survived beyond 5 years(7)
Perez-Montiel 2006 5 76 3/2 P(2)U(1)UP(2) T2(1)T3(2)T4(2) NU(5) all died 3-24mo latermetastasize to regional lymph node(2),bone(1)
Munakata 2007 1 73 0/1 P(1) T4(1) NU+C(1) 14 mo,died of metastases to the left adrenal gland and the regional lymph nodes
Guo 2009 11 64.2 9/2 P(5)U(4)UP(2) T2(2)T3(8)T4(1) NU+C(11) died of tumor(4)surviving but developed metastasis(4)alvie,no evidence of disease(3)
Cheng 2010 3 64.3 2/1 P(2)U(1) T1(1) T3(1)T4(1) NU(1)NU+C(2) 15mo,died(1)alvie,no evidence of disease(2)

*Age–Mean age; **M/F–Male/Female; #Site;U–ureter,P–renal pelvis,UP–ureter and pelvis; ^NU,Nephro-Ureterectomy; ^^R,Radiotherapy; ^^^C,Chemotherapy; ^^^^UR,Ureteral Resection; ¤No,not received surgery

Discussion 

Carcinoma with a micropapillary component was first described in 1982 as a variant of endometrial carcinoma initially designated as papillary serous carcinoma of the uterus[15]. Later, this subtype was found in thyroid[16],breast[17], and lung cancers[18]. In 1994, Amin et al first reported a series of 18 cases of a micropapillary variant of transitional cell carcinoma (TCC) and suggested that this particular pattern could be associated with a more aggressive course of the tumor than usual TCC [1] .

The incidence of this disease is reported as approximately 2.8% [9]. Most cases originated from the renal pelvis; 33 patients, (including our 3 cases);15 cases arose from the ureter [6,7,8,11], and five cases were reported to have tumor both in the ureter and the renal pelvis [6,10,12]. The overall male to female ratio was 2:1(35:17). In a study by Alvarado-Cabrero, the patient’s gender and age could not be established due to incomplete clinical information.  Clinical staging information and follow up information were not available for this patient. Mean age at diagnosis is 72.07 (range  22 to 92 years). MUC of the upper urinary tract may have similar etiological factors to that of conventional UC of the upper urinary tract. It is noteworthy that two out of three patients reported by Cheng et al are both residents of the area affected by the black foot endemic in Taiwan[13]. This area has been reported to have a high prevalence of invasive upper urinary tract tumors[19-21]

There is no specific feature that allows definite clinical diagnosis of MUC of the upper urinary tract. No specific symptoms for MUC of the upper urinary tract have been reported. Painless gross hematuria was the most common symptom. Another reported symptom was flank pain. In our study, two patients presented with hematuria, one presented with flank pain. The diagnosis of MUC is based on the presence of a secondary micropapillary bud in the stroma beneath the urothelium in microscopic studies[1]. In addition to urothelial carcinoma, a micropapillary pattern has been reported in carcinomas of the ovary, breast, lung, colon, and major salivary glands[22–27 ]. In all of these organs, the micropapillary pattern shares a distinct morphology that is characteristic of small infiltrating clusters or nests of tumor cells within lacunae. Therefore, it is important to exclude metastases with micropapillary features when making a definite diagnosis of primary tumor. Immunohistochemical stains are useful in identifying the primary tumor. Ramalingam et al reported an invasive micropapillary carcinoma of the breast metastatic to the urinary bladder[28]. The micropapillary components were morphologically identical in the breast, urinary bladder, and endometrium; however, the tumor cells from the breast, endometrium, and urinary bladder were positive for cytokeratin 7 and estrogen receptor and negative for cytokeratin 20.

Therapeutic modalities depend upon a tumour’s clinical stage. They include surgery, chemotherapy and radiotherapy. Nephroureterectomy (NU) or partial ureteric resection was curative in patients with low stage disease and in a minority of those with high stage disease. In a study by Vang et al, the patient with stage I was treated by laser ablation therapy. This case appears to be less invasive because of earlier attention and intervention, but the follow up information was incomplete, and the patient’s clinical course remains to be further defined[7]. In our study, one patient with stage II disease who received NU was alive with no evidence of disease. Extensive lymph node dissection may been curative. In a study by Holmäng, one patient with multiple MC lymph node metastases who received extensive lymph node dissection survived 78 months without evidence of recurrence[9]. In our study, one patient who received regional lymph node dissection died 6 months later. According to the study of Munakat et al, radiotherapy did not influence the course of disease[11]. Oh et al reported on a 79-year-old male patient with a mid ureteric tumor treated by nephroureterectomy. The histopathological examination showed periureteric fat invasion and lymph node metastases with a predominant MC pattern in 18 of 20 lymph nodes examined. The patient had local recurrence after one year and was treated with radiotherapy but died 8 months later of metastatic disease[6]. External beam radiotherapy also did not seem to have been of benefit in our patients. Although cisplatin, cyclophosphamide, doxorubicin, gemcitabine, methotrexate and vinblastin have been reported in various combinations, nothing was effective[29-32]. The response of MUC patients to chemotherapy seems to be poor. Cheng et al prescribed chemotherapy for one patient with metastatic lesions. However, the patients died 15 months after the diagnosis[13]. Munakata et al reported one case whose pathological stage was pT4N2M1, and who underwent surgery. Although she received chemotherapy, she died of her disease 14 months postoperatively[11]. In our study, one patient had metastases to the ipsilateral adrenal gland, he received LNU and adrenalectomy, systemic GC chemotherapy (gemcitabine and cisplatin) were given, but he relapsed after 6 months and died from metastases 12 months later. However, Holmäng et al reported one case who showed partial response to methotrexate + vinblastin + doxorubicin + cisplatin followed by gemcitabine [9]. To date, no reports have recommended any preferred chemotherapy for MUC.

The prognosis is poor since most patients with MPC of the upper urinary tract initially present with advanced disease. It demonstrated a high tendency to invade lymphovascular spaces and to metastasize to lymph nodes and other organs. In a letter to the editor of the American Journal of Surgical Pathology, Ribé describes a patient who had metastasis to the gastric mucosa and died one month later[5]. Holmang et al reported 26 cases of MUC from 18 hospitals. All patients presented with invasive disease. Twenty patients died of their disease [9]. In a study by Perez-Montiel, all patients had tumors that showed high grade histologic features, and most were in an advanced clinical stage. All patients in the study died of tumor from 3 to 24 months after the diagnosis[10]. Guo reported 11, eight and one patients presenting with stage III and stage IV disease, respectively. Four died of their tumor, of the seven surviving patients, four developed metastases to the lung, colon, or retroperitoneum[12]. In our study, two  of  three  patients presented with pT3 or pT4 tumors, and two of these patients died of their disease within one year of surgery. It is noteworthy that one of the two  patients who received LNU developed port-site metastasis. To the best of our knowledge, it is first reported case in the literature of laparoscopic port-site metastasis of MUC. Therefore, we should be careful when determining the indications for laparoscopic surgery in such patients.

In conclusion, MUC of the upper urinary tract which often presents at an advanced stage with lymphovascular invasion and distant metastasis is probably an underreported variant of urothelial carcinoma associated with poor prognosis. Clinically, MUC of the upper urinary tract is far more aggressive than conventional urothelial carcinoma of the upper urinary tract. Early diagnosis of MUC and an understanding of the  nature and biologic behaviour of this tumor have important clinical implications in treatment. Patients with MUC should be diagnosed promptly and treated aggressively. Surgery is curative in less advanced cases. However, radiotherapy and systemic chemotherapy, either alone or as part of combined therapy, appear to be ineffective. The optimal treatment strategy is yet to be defined. Meanwhile, how to diagnose this disease earlier needs to be further investigated.

References  

1.Amin MB,Ro JY,el-Sharkawy T,et al.Micropapillary variant of transitional cell carcinoma of the urinary bladder: histologic pattern resembling ovarian papillary serous carcinoma. Am J Surg Pathol.1994;18:1224-1232.

2.Johansson SL, Borghede G, Holmang S.Micropapillary bladder carcinoma: a clinicopathological study of 20 cases. J Urol. 1999;161:1798–1802.

3.Maranchie JK, Bouyounes BT, Zhang PL, et al. Clinical and pathological characteristics of micropapillary transitional cell carcinoma:a highly aggressive variant. J Urol. 2000;163:748-751.

4.Kamat AM, Dinney CP, Gee JR, et al. Micropapillary bladder cancer: a review of the University of Texas M. D. Anderson Cancer Center experience with 100 consecutive patients. Cancer. 2007;110:62–67.

5.Ribé A, Solé M, Campo E,et al.Papillary transitional cell carcinoma.Am J Surg Pathol. 1996 ;20:125-6.

6.Oh YL, Kim KR.Micropapillary variant of transitional cell carcinoma of the ureter.Pathol Int.2000;50:52-6.

7.Vang R, Abrams J.A micropapillary variant of transitional cell carcinoma arising in the ureter.Arch Pathol Lab Med.2000;124:1347-8.

8.Alvarado-Cabrero I, Sierra-Santiesteban FI, Mantilla-Morales A,et al.Micropapillary carcinoma of the urothelial tract. A clinicopathologic study of 38 cases.Ann Diagn Pathol. 2005;9:1-5.

9.Holmäng S, Thomsen J, Johansson SL.Micropapillary carcinoma of the renal pelvis and ureter.J Urol. 2006;175:463-6.

10.Perez-Montiel D, Hes O, Michal M,et al.Micropapillary urothelial carcinoma of the upper urinary tract: Clinicopathologic study of five cases.Am J Clin Pathol. 2006;126:86-92.

11.Munakata S, Tahara H, Kojima K,et al.Micropapillary urothelial carcinoma of the renal pelvis: report of a case and review of the literature.Med Sci Monit.2007;13:CS47-52.

12.Guo CC, Tamboli P, Czerniak B.Micropapillary variant of urothelial carcinoma in the upper urinary tract: a clinicopathologic study of 11 cases.Arch Pathol Lab Med. 2009;133:62-6.

13.Cheng YT, Luo HL, Sung MT,et al.Micropapillary variant of urothelial carcinoma: a report of 4 cases and literature review.Chang Gung Med J. 2010;33:461-5.

14.obin LH, Wittekind Ch: TNM classification of tumours of the urinary bladder.In TNM classification of malignant tumors Edited by:Sobin LH, Wittekind Ch. New York, NY: Wiley; 1997:29-31.

15.Hendrickson M, Ross J, Eifel P, et al.: Uterine papillary serous carcinoma: a highly malignant form of endometrial adenocarcinoma.  Am J Surg Pathol 1982, 6:93-108.

16.McDougall IR, Camargo CA: Treatment of Micropapillary Carcinoma of the Thyroid: Where Do We Draw the Line?  Thyroid 2007,17:1093-1096.

17.Siriaunkgul S, Tavassoli FA: Invasive micropapillary carcinoma of the breast. Mod Pathol 1993, 6(6):660-2.

18.Maeda R, Isowa N, Onuma H,et al.Lung adenocarcinomas with micropapillary components.Gen Thorac Cardiovasc Surg. 2009;57:534-9.

  1. Chiang PH, Huang MS, Tsai CJ, Tsai EM, Huang CH,Chiang CP. Transitional cell carcinoma of the renal pelvis and ureter in Taiwan. DNA analysis by flow cytometry.Cancer 1993;71:3988-92.

20.Kang CH, Yu TJ, Hsieh HH, et al.The development of bladder tumors and contralateral upper urinary tract  tumors after primary transitional cell carcinoma of the upper urinary tract. Cancer 2003;98:1620-6.

21.Chou YH, Huang CH. Unusual clinical presentation of upper urothelial carcinoma in Taiwan. Cancer 1999;85:1342-4.

  1. Haupt B, Ro JY, Schwartz MR, et al. Colorectal adenocarcinoma with micropapillary pattern and its association with lymph node metastasis. Mod Pathol.2007;20:729–733.
  2. Siriaunkgul S, Tavassoli FA.Invasive micropapillary carcinoma of the breast. Mod Pathol. 1993;6:660–662.

24.Amin MB, Tamboli P, Merchant SH, et al. Micropapillary component in lung adenocarcinoma: a distinctive histologic feature with possible prognostic significance. Am J Surg Pathol. 2002;26:358–364.

  1. Nagao T, Gaffey TA, Visscher DW, et al. Invasive micropapillary salivary duct carcinoma: a distinct histologic variant with biologic significance. Am J Surg Pathol. 2004;28:319–326.

26.Burks RT, Sherman ME, Kurman RJ. Micropapillary serous carcinoma of the ovary: a distinctive low-grade carcinoma related to serous borderline tumors.Am J Surg Pathol. 1996;20:1319–1330.

27.Kim MJ, Hong SM, Jang SJ, et al. Invasive colorectal micropapillary carcinoma: an aggressive variant of adenocarcinoma. Hum Pathol. 2006;37:809–815.

28.Ramalingam P, Middleton LP, Tamboli P,et al.Invasive micropapillary carcinoma of the breast metastatic to the urinary bladder and endometrium: diagnostic pitfalls and review of the literature of tumors with micropapillary features.Ann Diagn Pathol.2003;7:112-9.

29.Regalado JJ: Mixed micropapillary and trophoblastic carcinoma of bladder: report of a first case with new immunohistochemical evidence of urothelial origin. Hum Pathol, 2004; 35: 382–84

30.Sugino Y, Negoro H, Iwamura H et al: Micropapillary variant of transitional cell carcinoma of the bladder. Int J Urol. 2004 Sep;11(9):792-4.

31.Nishizawa K, Kobayashi T, Mitsumori K et al: Micropapillary bladder cancer. Int J Urol, 2005; 12: 506–8

  1. Dhouib RS, Abbes I, Mrad K et al: Micropapillary transitional cell carcinoma of the urinary bladder. Report of two cases. Pathologica, 2005; 97: 338–40

 

Date added to bjui.org: 25/02/2012
DOI: 10.1002/BJUIw-2011-121-web

 

Primary and Secondary Mesothelioma of the Tunica Vaginalis: a comparative case study

/by

We report two cases of mesothelioma of the tunica vaginalis, one primary and one secondary, both with a history of possible exposure to asbestos and discuss their pathogenesis, diagnosis and management.

 

Authors: Swetha Vijayan1; Richard Carr2; John Strachan

1. Senior House Officer, Department of Urology, South Warwickshire NHS Foundation Trust, Warwick, United Kingdom
2. Consultant Histopathologist, Department of Histopathology, South Warwickshire NHS Foundation Trust, Warwick, United Kingdom
3. Consultant Urologist, Department of Urology, South Warwickshire NHS Foundation Trust, Warwick, United Kingdom

Corresponding Author: Swetha Vijayan, Department of Urology and Department of Histopathology, South Warwickshire NHS Foundation Trust, Warwick, United Kingdom. Email: [email protected]

 

Abstract
We report two cases of mesothelioma of the tunica vaginalis, one primary and one secondary, both with a history of possible exposure to asbestos and discuss their pathogenesis, diagnosis and management.
The first patient is an 89 year-old man referred with recurrent left scrotal swelling following repeated fluid aspirations. His only exposure to asbestos had been through his son who had died one year previously of asbestosis.  At hydrocoelectomy, a thickened tunica vaginalis and heavily blood stained fluid were noted.  Histopathology and immuno-staining confirmed malignant mesothelioma and urgent radical orchidectomy was performed. The tumour recurred within a month with spread to mediastinal and para-aortic lymph nodes.
The second patient is a 68 year-old man referred with increasing shortness of breath and pain over the left side of his chest. He was a retired brick layer with possible occupational exposure to asbestos. Investigations and biopsy established a diagnosis of pleural mesothelioma.  During evaluation for chemotherapy, he presented with a hard testicular lump and underwent radical orchidectomy. Histology confirmed the presence of mesothelioma involving the tunica vaginalis. Follow-up CT scan showed no abdominal or pelvic disease.

 

Introduction
Malignant mesothelioma of the tunica vaginalis is an uncommon, locally aggressive tumour, with over 200 cases reported in the literature to date [1]. The disease usually presents in men over 50 years of age, though, there have been cases reported in younger age groups [2] [3]. Exposure to asbestos has been considered as a risk factor. Patients usually present with a hard testicular lump or scrotal swelling. However, the disease lacks characteristic symptoms and signs so that in most cases the diagnosis is made following surgical exploration.  Mesothelioma of the tunica vaginalis has a poor prognosis and high rate of recurrence and nodal metastases. Here we present two cases of malignant mesothelioma of the tunica vaginalis, one primary and one secondary, both associated with a history of possible exposure to asbestos.

 

Case 1
An 89 year old man was referred with a 6-month history of swelling in the left hemi-scrotum. The referring general practitioner, suspecting a hydrocoele, aspirated fluid on two occasions but the swelling soon returned. On examination there was a trans-illuminating large left sided hydrocoele. At elective hydrocoelectomy, we found heavily blood stained fluid and a thickened hydrocoele wall. Microscopic examination of the surgical resection specimen revealed fibrous tissue infiltrated by acinar and papillary structures (Fig. 1).

 

Figure 1. Microscopic examination of the surgical resection specimen

 

The differential diagnosis included adenocarcinoma and mesothelioma.  Immunohistochemistry supported a final diagnosis of malignant mesothelioma as follows: positivity for calretinin (Fig 1 inset), epithelial membrane antigen (EMA), cytokeratin (CK)20,  and negative for CK5, CK6, CEA, BerEP4, thyroid transcription factor 1 (TTF1), oestrogen receptor, thyroglobulin and prostate specific antigen.

 

The patient underwent radical orchidectomy with wide local excision. Pathological examination confirmed mesothelioma of the tunica vaginalis extensively infiltrating the epididymis (Fig. 1A) and the testicular parenchyma with vascular invasion.  Tumor seedlings were also noted in the dermal scar tissue. Following the diagnosis, further questioning revealed no personal history of direct asbestos exposure.  However, the patient had been living with his son who had occupational asbestos exposure and had died of asbestosis one year prior to this presentation.

 

One month later the patient’s wound broke down and on examination nodules were palpable in the wound. A further biopsy confirmed locally recurrent disease. CT scan of the chest and abdomen showed no evidence of pleural or peritoneal tumour, but identified significant mediastinal and left sided para-aortic lymph node disease without any pelvic lymphadenopathy. He declined further active management and died within a few months.

 

Case 2
A 68 year old retired man was referred by the general practitioner to a respiratory physician, with a history of increasing shortness of breath and fatigue for 2 months. The patient also reported left sided lower chest pain and mild cough. There was no history of haemoptysis, weight loss or night sweats. He was an ex-smoker (for 30 years).  There was a possibility of asbestos exposure during his former job as a brick layer. On auscultation, there was decreased air entry on the left side of his chest.  Chest X-ray showed a large left sided pleural effusion. An inter-costal tube was placed which drained 6L of thick haemorrhagic fluid over an 11-day period with a good clinical symptomatic response. CT scan of his thorax initially showed no underlying lung or pleural pathology. Pleural fluid cytology was reported as showing individual, dispersed, reactive mesothelial cells, abundant neutrophils and macrophages, but no malignant cells. Subsequent MRI scan also revealed no suggestion of a neoplastic process. Suspecting infection, he was treated with antibiotics and discharged with close follow-up.

 

Follow-up chest X-ray 6-months later showed some increased markings present locally in the left lower zone possibly indicating focal inflammatory changes. Within the next 3-months, there was a recurrence of his symptoms and the patient was referred to cardiothoracic surgery.  A video assisted thoracoscopic examination was suspicious of pleural mesothelioma and a biopsy was taken. Histology confirmed the diagnosis of an epithelioid malignant mesothelioma.

 

During his preparations for chemotherapy, the patient was referred to urology with the recent onset of a swelling in his right testicle. On examination, the left testis was normal, but the right testis was rock hard. An ultrasound scan showed a solid, low echogenicity, 3.5cm mass within the right inguinal canal that appeared to be arising from the right epididymis. A radical orchidectomy was performed. The histopathology revealed malignant mesothelioma of tunica vaginalis (Fig. 2) enveloping the testis, epididymis and spermatic cord up to and including the proximal resection margin.

 

Figure 2. Histopathology results

A follow-up CT scan of the thorax, abdomen and pelvis showed significant deterioration in the pleural lesion but no other extra-thoracic abnormality. The patient died of progressive pleural disease 2 years from the first referral (10 months after his radical orchidectomy).  There is no information regarding local recurrence of the intra-scrotal disease.

 

Discussion 
Malignant mesothelioma arises from the mesothelial cells lining the pleural, peritoneal, and pericardial cavities and rarely the tunica vaginalis. Pleural mesothelioma is by far the most common form of mesothelioma and has a well-recognised link to asbestos exposure [4].  Mesothelioma of the peritoneum or tunica vaginalis is rare in the absence of pleural disease but peritoneal involvement may present with spread to the tunica vaginalis because the latter develops as a direct extension of the peritoneal mesothelium. During the embryonic period all serous cavities develop from a common coelom and are continuous until partitions separate them. Malignant cells may track down from the pleural cavities via persisting connections between the body cavities or by direct extension of disease through the diaphragm. In our second patient, who developed overt pleural disease and spread to tunica vaginalis without any abdominal lesion, we presume this was the case.
Asbestos exposure, trauma and chronic hydrocoele have all been implicated as risk factors in the development of paratesticular mesothelioma [2] [5]. A few cases have also been reported with no prior associated disease process [6].  Due to the rarity of primary tunica vaginalis mesothelioma, little is known of its pathogenesis. In patients with asbestos exposure, we speculate that the asbestos fibres may collect in the tunica vaginalis via the coelomic connections described earlier. A small number of fibres may also reach the area via the blood stream after being absorbed from the lungs and gastrointestinal tract. Asbestos exposure mainly occurs from breathing in asbestos fibres. When products containing asbestos are disturbed, they release fibres into the air which may be inhaled [7]. Oral and dermal exposures have also been described [8]. Familial occupational exposure may also lead to indirect exposure via contaminants brought home. Plas et al reported in their study that a familial occupational history to asbestos increased the risk of malignant mesothelioma of tunica vaginalis 10-fold [2]. The relationship between asbestos exposure and mesothelioma is well established but the mechanisms underlying tumourogenesis are not fully understood. Chronic irritation leading to a hyperplastic inflammatory response along with oncogenic mutations caused by the asbestos fibres may eventually lead to tumour development [4]. We consider our first case to be a presentation of primary tunica vaginalis mesothelioma without pleural or peritoneal disease.  The mediastinal and para-aortic lymph node disease are compatible with metastases from the scrotal disease.  The potential source of asbestos exposure was his son who had died one year earlier of asbestosis and who could have brought contaminants home through his work clothes thirty years earlier.
Mesothelioma presenting in the tunica vaginalis usually affects men between 50 to 70 years of age. The disease presents as a scrotal swelling or hard testicular mass but is rarely diagnosed pre-operatively [2]. The histopathological diagnosis of malignant mesothelioma can be challenging and should be supported by immunohistochemical studies. Winstanley et al studied the immunohistochemical profile of 20 testicular malignant mesotheliomas finding the following positivity rates: calretinin and EMA 100%; thrombomodulin 89%; CK7 83%: CK5/6 72% but only 11% were positive for BerEp4. All cases were negative for CK20 & CEA [9]. Calretinin is a vitamin D dependent calcium binding protein, detected in most malignant mesotheliomas, and considered one of the most specific markers for epithelioid mesothelioma [10]. The main differential diagnoses for mesotheliomas are rare primary adenocarcinomas of the rete testis and metastatic adenocarcinomas that are calretinin and CK5/6 negative and usually BerEP4 and CEA positive.
In any case of confirmed mesothelioma of the tunica vaginalis, radical orchidectomy remains the treatment of choice. Adjuvant radio- or chemotherapy is advised during initial management but radiotherapy is considered to be more effective [2]. In 1998, Plas et al reviewed 73 cases of testicular mesothelioma and found 37.5% developed tumour recurrence following local resection of the hydrocoele wall while only 10.5% did so after scrotal orchidectomy and 11.5% after inguinal orchidectomy [2]. The median survival of patients was reported to be 23 months. The risks of recurrence following radical treatment is maximal during the first two years and close monitoring and follow-up is therefore required [2]. Malignant mesothelioma is often extensive at presentation with a high incidence of rapid local progression and metastatic spread as in our two patients.

 

Conclusion
Primary or secondary mesothelioma of the tunica vaginalis is extremely rare in occurrence. Preoperative diagnosis is difficult due to the rarity of the disease and the non-specific clinical presentations. A high index of suspicion is required in all patients with scrotal swellings and known exposure to asbestos. Increased awareness of the condition might help in improving the pre-operative diagnosis. The tumour has a rapidly progressive course with a high recurrence rate and poor prognosis. Early diagnosis, vigorous management and close follow-up may provide a better outcome and may improve disease free survival.

 

References
1. Bisceglia M, Dor DB, Carosi I, Vairo M, Pasquinelli G. Paratesticular mesothelioma: Report of a case with comprehensive review of literature. Adv Anat Pathol 2010. 17:53-70.
2. Plas E, Riedl CR, Pflunger H; Malignant mesothelioma of the tunica vaginalis testis: review of the literature and assessment of prognostic parameters. Cancer 1998. 83:2437-2446.
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4. Cancer Research UK. Mesothelioma: Risks and Causes. https://cancerhelp.cancerresearchuk.org/type/mesothelioma/about/mesothelioma-risks-and-causes
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7. Agency for Toxic Substances and Disease Registry. What is Asbestos? Retrieved April 10, 2009 from: https://www.atsdr.cdc.gov/asbestos/more_about_asbestos/what_is_asbestos/
8. Agency for Toxic Substances and Disease Registry. Toxicological Profile for Asbestos. September 2001. Retrieved April 10, 2009 from: https://www.atsdr.cdc.gov/toxprofiles/tp61.pdf
9. Winstanley AM, Landon G, Berney D, Minhas S, Fisher C, Parkinson MC. The immunohistochemical profile of malignant mesotheliomas of the tunica vaginalis: a study of 20 cases. Am J Surg Pathol 2006. 30:1-6
10. Candura SM, Canto A, Amatu A, Gerardini M, Stella G, Mensi M, Poggi G. Malignant mesothelioma of the tunica vaginalis testis in a petrochemical worker exposed to asbestos. Anticancer Research 2008 Mar-Apr; 28(2B):1365-68.

Date added to bjui.org: 23/02/2012

DOI: 10.1002/BJUIw-2011-127-web

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