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Adult-type testicular granulosa cell tumor: a case report and radiological findings of a rare testicular tumor

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We report a case of an adult-type testicular granulosa cell tumor and the radiological findings of this rare tumor.

Authors: Kobayashi, Ko; Itoh, Naoki; Sakai, Shigeru; Sato, Masaaki
Corresponding Author: Kobayashi, Ko

 

Abstract
Among testicular neoplasms, adult-type testicular granulosa cell tumors are quite rare. We report a case of an adult-type testicular granulosa cell tumor and the radiological findings of this rare tumor. The patient was a 49-year-old man with a mass on the left testis that grew slowly for 5 years. An ultrasound scan of the left testis showed a hypoechoic and well-circumscribed mass within the normal testicular parenchyma. A computer tomographic (CT) scan of the whole abdomen revealed the mass to be mildly ring-enhanced in the left testis after contrast administration. We performed left radical orchiectomy. The pathological findings of the surgical specimen showed that the tumor was consistent with an adult-type granulosa cell tumor. The patient has no evidence of disease after 24 months of follow-up.

Introduction
Sex cord-stromal tumors account for only about 4% of testicular neoplasms [1]. Among such tumors, the adult-type testicular granulosa cell tumor is quite rare [1-3]. Therefore, radiological findings of this uncommon tumor have rarely been reported. We report a case of an adult-type testicular granulosa cell tumor including the radiological findings.

Case report
A 49-year-old man with a mass of the left testis that had grown slowly for 5 years visited our office in November 2009. We found a hard solid mass of his left testis on physical examination. An ultrasound scan of the left testis showed a hypoechoic and well-circumscribed mass within the normal testicular parenchyma (Figure 1a). A computer tomographic (CT) scan of the whole abdomen revealed the mass to be mildly ring-enhanced in the left testis after contrast-agent administration (Figure 1b). There were no enlarged lymph nodes or abnormalities in other organs in the CT findings. Tumor markers such as alpha-fetoprotein and human chorionic gonadotropin were within normal ranges. We diagnosed his scrotal mass as testicular tumor and performed left radical orchiectomy. The cut surface of the orchiectomy specimen displayed a solid, hard and white tumor occupying part of the testicular parenchyma. The size of the tumor was 2.6×2.5×2.0 cm. Microscopically, tumor cells had grooved nuclei (coffee-bean nuclei) and formed microfollicular, macrofollicular and trabecular patterns (Figure 2a). In the follicles, Call-Exner-like bodies were focally seen (Figure 2b). Immunostaining tests were positive for vimentin, CD99 and alpha-inhibin. These pathological findings showed that the tumor was consistent with an adult-type granulosa cell tumor. The patient has no evidence of disease after 24 months of follow-up.

Discussion
Granulosa cell tumors are classified under the category of sex-cord stromal tumors. Sex-cord stromal tumors account for only 4% of testicular neoplasms [1]. Granulosa cell tumors of the testis are morphologically similar to their ovarian counterparts. Two variants are distinguished, the adult and juvenile types [1-3]. Granulosa cell tumors of the ovary account for less than 5% of all malignant tumors but represent the most common malignant sex-cord stromal tumor of the ovary [4]. On the other hand, adult-type testicular granulosa cell tumors are quite rare [1-3,5].
Adult-type testicular granulosa cell tumors are mostly benign; however, malignant behavior is a possibility [1,2,5]. In a previous report, there was a case that metastasized to bone, presenting 6 years after orchiectomy [6]. Therefore we should perform long-term follow-up for our case.
In a previous case report, an ultrasound scan of a juvenile-type testicular granulosa cell tumor showed a well-defined multicystic intratesticular solid mass [7]. The present adult-type case was different in that it was hypoechoic and there was a well-circumscribed mass within the normal testicular parenchyma. Another report also showed a hypoechoic mass on the ultrasound scan of a patient with an adult-type testicular granulosa cell tumor [8]. Thus, these two variants had different findings on ultrasound scans. However, differential diagnosis of the classification of testicular neoplasms is difficult using only ultrasound scanning because approximately 80% of testicular neoplasms have a hypoechoic appearance on ultrasound scans [9].
Here we reported CT findings of a testicular tumor diagnosed as an adult-type granulosa cell tumor. To our knowledge, the current report is the third one with CT findings of the primary site of this quite rare testicular tumor. In the previous cases, there was one report of a mildly enhanced heterogenous soft tissue mass involving the testis and another with an enhanced mass in the scrotum on the CT scan [10-11]. Granulosa cell tumors of the ovary show a spectrum of imaging manifestations due to their various histologic appearances and arrangements of tumor cells [4]. Although the findings for this rare tumor on CT scans were slightly different in the three reports, it is unknown at this stage whether adult-type testicular granulosa cell tumors also present such a spectrum of imaging manifestations. We believe that accumulating knowledge from radiological findings on this rare testicular tumor will be useful for diagnosis of the disease in the future.

073fig1

 

 

 

 

 

 

 

 

 

 

Figure 1. Radiological findings.
(a) Ultrasound scan of the left testis. The black arrow indicates a hypoechoic and well-circumscribed mass within the normal testicular parenchyma.
(b) A computer tomographic scan shows a mass (white arrow) that is mildly ring-enhanced in the left testis after contrast-medium administration.

073fig2

 

 

 

 

 

 

 

 

 

 

Figure 2. Pathological findings.
(a) Black circles indicate tumor cells with grooved nuclei (coffee-bean nuclei).
(b) White circles indicate Call-Exner-like bodies.

Conflicts of interest
The authors have nothing to disclose.

References
1. Ulbright TM. Neoplasms of the testis. In: Bostwick DG, Eble JN (eds). Urologic Surgical Pathology. Mosby, St. Louis, 1997; 567-646.
2. Eble JN, Sauter G, Epstein JI, Sesterhenn IA. Pathology and Genetics of Tumors of the Urinary System and Male Genital Organs. IARCPress, Lyon, 2004.
3. Ulbright TM, Amin MB, Young RH. Tumors of the Testis, Adnexa, Spermatic Cord, and Scrotum. American Registry of Pathology, Washington, D.C.,1999.
4. Jung SE, Rha SE, Lee JM et al. CT and MRI findings of sex cord-stromal tumor of the ovary. Am J Roentgenol. 2005; 185: 207-15.
5. Hammerich KH, Hille S, Ayala GE et al. Malignant advanced granulosa cell tumor of the adult testis: case report and review of the literature. Hum Pathol. 2008; 39:701-5.
6. Suppiah A, Musa MM, Morgan DR, North AD. Adult granulosa cell tumor of the testis and bony metastasis. A report of the first case of granulosa cell tumor of the testicle metastasising to bone. Urol Int. 2005; 75:91-3.
7. Lin KH, Lin SE, Lee LM. Juvenile granulosa cell tumor of adult testis: a case report. Urology. 2008; 72: 230.e11-3.
8. Guzzo T, Gerstein M, Mydlo JH. Granulosa cell tumor of the contralateral testis in a man with a history of cryptorchism. Urol Int. 2004; 72:85-7.
9. Heiken JP. Tumors of the testis and testicular adnexa. In: Pollack HM, McClennan BL (eds). Clinical Urography. W.B. Saunders Company, Philadelphia, 2000; 1716-1742.
10. Gupta A, Mathur SK, Reddy CP, Arora B. Testicular granulosa cell tumor, adult type. Indian J Pathol Microbiol. 2008; 51: 405-6.
11. Song Z, Vaughn DJ, Bing Z. Adult type granulosa cell tumor in adult testis: report of a case and review of the literature. Rare Tumors. 2011; 3: e37.

 

Date added to bjui.org: 11/02/2013

DOI: 10.1002/BJUIw-2012-073-web

 

Failed salvage of late presentation adult testicular torsion: cases discovered on serial testicular scintigraphy

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Here we report two cases of late presentation adult testicular torsion in which the patients had undergone a failed initial salvage trial, in which final decision for surgery were made on the basis of serial testicular scintigraphy. 

Authors: Young Hwii Ko,1 Gi Joeng Cheon,2 Tae Young Park,1 Sung Gu Kang,1 Du Geon Moon,1 Jun Cheon,1 Jeong Gu Lee,1 Je Jong Kim1

1. Department of Urology, Korea University School of Medicine, Seoul 136-705, Korea
2.  Department of Nuclear Medicine, Korea University School of Medicine, Seoul 136-705, Korea

Corresponding Author: Je Jong Kim, MD, PhD, Professor, Department of Urology, Korea University School of Medicine,  Anam-dong 5-ga, Seongbuk-gu, Seoul 136-705, Korea.    E-mail: [email protected]

 

 

Abstract
Although successful manual detorsion relieves the acute symptoms of testicular torsion, which has traditionally been regarded to be rare in adulthood, about one-third of patients had been reported to have residual symptoms. In case of initial surgical exploration, the appearance of the testicle is the sole criterion in making a decision regarding testis removal or retention. Hence, serial radiologic workups after an initial salvage trial may provide an objective evaluation, enabling an informed surgical decision to be made for failed initial salvage cases. Here, we describe two cases of late presentation adult testicular torsion, in which the absence of normal blood flow was diagnosed by follow-up serial testicular scintigraphy, after an initial trial to salvage the affected testis by manual or surgical detorsion.

 

Introduction
The diagnosis of testicular torsion is made routinely with the aid of radiologic modalities including Doppler ultrasonography (US) or radionuclide imaging [1], in addition to the presence of typical symptoms concomitant with loss of the cremasteric reflex. Following diagnosis, urgent or semi-elective surgical exploration with/without manual detorsion can be tried as an initial attempt to salvage the testis. During surgery, the testis is observed for any improvement in color. A decision to remove or retain the affected testis is subjective and, although based on the appearance of the testis, is also likely to be influenced by the age of the patient and the degree and duration of torsion [2]. Using manual reduction alone, while successful untwisting relieves the acute symptoms, the repeat torsion rate can be 27.7% to 32% [3, 4]. Thus, serial radiologic evaluation after an initial salvage trial may provide objective evidence for the viability of the affected testis, in addition to being effective as an initial evaluation modality. For this purpose, radionuclide imaging may provide an additional advantage over US, avoiding operator-dependency, provided it can be conducted without delay.
Here we report two cases of late presentation adult testicular torsion in which the patients had undergone a failed initial salvage trial, in which final decision for surgery were made on the basis of serial testicular scintigraphy.

 

Case Report 1
A 49-year-old male presented to the outpatient department of our institute with acute pain in the left inguinal area and scrotum, which had begun 3 days previously. On physical examination, both testes were located normally in the scrotum, with tenderness only in the left testis with mild scrotal swelling. Urinalysis revealed no pyuria, but the cremasteric reflex on the left was reduced compared with that on the right side. US revealed maintained left testicular blood flow with mild enlargement of the epididymis. With the suspicion of testicular torsion, testicular scintigraphy using technetium 99m pertechnetate was performed. A definite photon defect was evident in the left testis with increased uptake in the surrounding area (Figure 1a).

 

Figure 1a. Initial testicular scintigraphy. Arrowhead indicates photon defect area in left testis with increased surrounding areas.

 

 

Then, as initial management, manual untwisting to the clockwise direction was tried at once in the outpatient setting, after which tenderness and left scrotal pain was abruptly decreased. Considering residual torsion concomitant with underline deformity in both scrotums, follow-up testicular scintigraphy and elective surgery were planned. Testicular scintiography taken the day after manual reduction revealed no interval change compared to the image recorded before manual reduction, regardless of improvement in symptom (Figure 1b).

 

Figure 1b. Follow-up testicular scintigraphy after manual detorsion on left testis. The photon defect area (arrowhead) showed no significant interval change compared with prior imaging.  

 


 

Elective surgical exploration revealed 180º counterclockwise torsion of left testis with partial bell-clapper deformity, then bilateral orchiopexy was performed. Follow up imaging taken the day after surgical correction showed significant improvement in photon defect (Figure 1c).

 

Figure 1c. Follow up testicular scintigraphy taken the day after surgical correction showed significant decreased in photon defect area (arrowhead).

 


 

 

Case Report 2  
 

 

A 23-year-old male presented to our emergency department complaining of abrupt development (11 hours previous) of left scrotal pain and swelling. Urinalysis revealed pyuria with 10-29 leukocytes in each high power field. US performed in the emergency setting showed decreased blood flow in left scrotum, with mildly enlarged epididymis. Under suspicion of testicular torsion, surgical exploration was performed 13 hours after the onset of pain. Testicular scintigraphy taken immediately before the operation confirmed a photon defect in left scrotum (Figure 2a).

 

Figure 2a. Initial testicular scintigraphy. The area for left scrotum showed no perfusion (arrowhead). 

 


 

In the operative field, a 360º counterclockwise torsion was found. After untwisting of the testis and soaking in warm saline for 10 minutes, return of fresh color on surface of the tunica albuginea was observed, with fresh bleeding upon a tentative small incision (Figure 2b).

 

Figure 2b. At surgical exploration, return of normal color with fresh bleeding upon a tentative small incision was observed (arrowhead).

 


Based on these findings, bilateral orchiopexy was performed. However, the patient still complained of a similar degree of scrotal pain one day after the operation. A follow-up testicular scintigraphy was performed, which revealed a perfusion defect on the left testis (Figure 2c)

 

Figure 2c. Follow-up imaging after orchiopexy. While increased compared with prior imaging, the scintigraphy still showed obvious photon defect with increased surrounding areas (arrowhead).

 

 

In a following orchiectomy the day after the initial operation, necrosis of whole left testis was revealed (Figure 2d). An orchiectomy concomitant with prosthesis insertion was conducted.

 

Figure 2d. Final specimen revealed totally necrotic change of left testis.

 

 

Discussion

 

Acute scrotal pain remains the most important differential diagnosis requiring exclusion or prompt management among urologic emergencies, as missed or delayed diagnosis of testicular torsion can lead to organ loss, cosmetic deformity, and compromised fertility. Although testicular torsion in adulthood is thought to be relatively unusual in adults, it may occur at any age. An estimated 39% of all cases of torsion develop in adulthood [5], including men in the sixth and seventh decades of life. The primary goal in management of testicular torsion is testicular salvage, with the goal of maintaining fertility. To achieve this, there is approximately a 4-8 hour window from the onset of torsion symptoms until surgical intervention is required to save the affected testis. Delay in diagnosis and subsequent delay in surgery risk testicular viability, with nearly 80% of affected testes infarcted after 10 hours from the onset of pain, and after 24 hours nearly 100% are infarcted and non-salvageable [6].
Age has been suggested as a variable effect on testicular salvage rate. Comparison with younger counterparts revealed that the reported testicular salvage is generally poor in adulthood [1, 7]. This likely is due to a lack of recognition of the potential for adult torsion by physicians, as well as differences in the severity of spermatic cord twisting in adults versus children [4]. A recent examination of 2248 men diagnosed with testicular torsion using a multivariate model estimating the probability of orchiectomy showed that only age was significant variable [8]. In the study, the prevalence of testicular torsion was 19% among those aged 1-9 years, 33% among those aged 10-17 years, and 41% among those aged 18-25 years. For every year increase in age, the adjusted odds of having an orchiectomy increased by 1.08 (95% CI, 1.03-1.13), or an increase of 8% in the odds per year.
The exceeded prevalence of testicular torsion in adults with higher orchiectomy rate indicates the importance of maintaining a high index of suspicion in acute scrotum symptoms in adulthood. After an initial trial to salvage the testis, the outcome should be monitored with care, as shown in these cases. While manual untwisting may allow prompt reperfusion of the testis, the resolution of symptoms does not necessarily correlate with the presence or absence of persistent torsion, because the testis may still be twisted, although to a lesser degree [3, 4]. Hence, radiologic evaluation should accompany this maneuver to confirm satisfactory detorsion. In case of surgical exploration, attempts should be made to salvage the testis if there is any sign of reperfusion after detorsion [2]. However, we believe that successful return of normal blood flow should also be followed by radiologic evaluation postoperatively, due to absence of reliable objective criteria at time of surgical decision. Regarding radiologic modality for follow up imaging, we chose testicular scintigraphy instead of US, which was presently performed as an initial radiologic modality upon suspicion of testicular torsion, presuming this approach to be more accurate [9] and objective, regardless of operator skill [10]. Actually, the blood flow detected by US was maintained in case 1, but the scintigraphy revealed a definite photon defect. Particularity in a follow-up setting, it can be performed electively. In this context, serial testicular scintigraphy after initial salvage trial to obtain objective evidence of procedural outcome enables adequate surgical decision for failed salvage cases.

 

Conclusions
In case of acute scrotal pain in adult, the applications of serial radiologic evaluation using testicular scintigraphy not only increase accuracy in diagnosis of testicular torsion, but also provide reliability on the outcome of initial salvage trial, enabling correct surgical approach.

 

References
1. Jaison A, Mitra B, Cameron P, Sengupta S. Use of ultrasound and surgery in adults with acute scrotal pain. ANZ J Surg. 2011; 81: 366-70.
2. Julia S. Barthold. Abnormalities of the testis and scrotum and their surgical management; in Campbell-Walth Urology 10th edition.  Editors Louise R. Kavoussi, Andrew C. Novick, Alan W. Partin, Craig A. Peters. Elsevier Sounders, 2011, Philadelphia, pp3590
3. Jefferson RH, Pérez LM, Joseph DB. Critical analysis of the clinical presentation of acute scrotum: a 9-year experience at a single institution. J Urol. 1997; 158 :1198-200.
4. Sessions AE, Rabinowitz R, Hulbert WC, Goldstein MM, Mevorach RA. Testicular torsion: direction, degree, duration and disinformation. J Urol. 2003; 169: 663-5.
5 Lee LM, Wright JE, McLoughlin MG. Testicular torsion in the adult. J Urol. 1983; 130: 93-4.
6. Davenport M. ABC of general surgery in children. Acute problems of the scrotum. BMJ. 1996; 312: 435-7.
7. Cummings JM, Boullier JA, Sekhon D, Bose K. Adult testicular torsion. J Urol. 2002; 167: 2109-10.
8. Mansbach JM, Forbes P, Peters C. Testicular torsion and risk factors for orchiectomy. Arch Pediatr Adolesc Med. 2005; 159: 1167-71.
9. Wu HC, Sun SS, Kao A, Chuang FJ, Lin CC, et al. Comparison of radionuclide imaging and ultrasonography in the differentiation of acute testicular torsion and inflammatory testicular disease. Clin Nucl Mel. 2002; 27: 490-3.
10. Ringdahl E, Teague L. Testicular torsion. Am Fam Physician. 2006; 74: 1739-43.

 

Date added to bjui.org: 15/07/2012
DOI: 10.1002/BJUIw-2011-128-web

 

Segmental testicular infarction mimicking a testis tumor in a 64-year-old man

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We report a case in which a 64-year-old man presented with a painful left hemiscrotum and focal mass in the left testis that mimicked a testicular tumor.

Authors: R.A.L. Jacobs1, E. Degreef2, K. De Laet1

1. Department of Urology, Maxima Medical Center, Veldhoven, The Netherlands
2. Department of Pathology and Medical Microbiology (PAMM), Eindhoven, The Netherlands

Corresponding Author: R.A.L. Jacobs, Department of Urology, Maxima Medical Center, Veldhoven, The Netherlands.Email: [email protected]

Introduction
Segmental testicular infarction (STI) is an uncommon testicular condition. We report a case in which a 64-year-old man presented with a painful left hemiscrotum and focal mass in the left testis that mimicked a testicular tumor.

 

Case report
 
A 64-year-old man with no relevant medical history presented to his general practitioner with acute left hemiscrotal pain and slight swelling of the left epididymis. He was treated with antibiotics for acute epididymitis. One week later, the patient was referred to the urology department because of persistent left sided scrotal pain.
There was no history of trauma and the patient experienced no fever or chills. He had not noted dysuria, hematuria or lower urinary tract symptoms. In the previous few months he had lost 11 kilograms in weight, which according to the patient, was due to his son’s death from melanoma.
Clinically, both testicles were normally positioned. The scrotal skin was devoid of erythema or thickening. The right testicle and epididymis were normal and non tender. The left epididymis was tender to palpation, but not enlarged. A firm non tender mass was palpated deep in the left testis. No pathological supraclavicular lymph nodes, abdominal masses or gynaecomastia were detected.
Ultrasonography revealed a heterogenous hypoechogenic oval shaped lesion in the upper third of the left testis with a size of 9.6mm x 1.8mm, suspicious of a tumor. Color Doppler ultrasound showed an avascular central zone in the lesion (Figure 1).

 

Figure 1. Color Doppler ultrasound showed an avascular central zone in the lesion
a) 

 

echo testis 1b) 
echo testis 2
Several hypoechogenic spots were observed, adjacent to the lesion. There were no signs of invasion of the tunica vaginalis. The right testis and both epididymes were normal on ultrasound.
Tumor markers for testicular cancer were within their normal ranges: αFP 3.3 μg/l (normal <7 μg/L), β-HCG <0.10 IU/l (normal: <3 IU/l) and LDH 240 U/l (normal: <248 U/l). The white blood cell count was 7.8.109/l (normal range: 4.0-10.0.109/l), creatinine 67 μmol/l  (normal 64-104 μmol/l), hemoglobin 10.1 mmol/l (normal 8.5-11.0 mmol/l) and hematocrit was 0.46 l/l (normal 0.40-0.50 l/l). Dipstick analysis of the urine was normal. A CT-scan of the chest and abdomen did not reveal any abnormalities.
Based on the clinical picture and ultrasound findings, a testicular tumor was suspected and a left inguinal exploration was performed. Correction of a small left indirect inguinal hernia was performed simultaneously. At exploration, the left testis appeared normal. No decolorization was noticed. A firm mass deep in the parenchyma was palpable. An inguinal orchiectomy was then performed. No complications occured and the patient made an uneventful recovery.
Pathological examination revealed an area of extensive infarction of the seminiferous tubules. In the surrounding testicular tissue no signs of intraepithelial germ cell neoplasia were present (Figure 2).

 

Figure 2. No signs of intraepithelial germ cell neoplasia presentT11-39855

 

Discussion
 
Unlike global testicular infarction, segmental testicular infarction (STI) is an uncommon testicular condition resulting from partial ischemia of the testis. STI commonly affects males between the second and fourth decades of life.1 However, several cases of STI in children have been reported.[1-3]
STI usually presents with acute scrotal pain and may resemble orchido-epididymitis or torsion of the spermatic cord. The condition can, however, present without pain. Sometimes swelling of the testis is present. In early stages palpation of the testis is normal, however, in more advanced cases an indurated testicular mass can be detected.[4]
Partial ischaemia of the testis is thought to be secondary to venous thrombosis, for which various local and systemic disorders have been reported as causal factors. Such factors include sickle cell anemia, polycythemia, vasculitis, iatrogenic vascular injury, trauma, arterial intimal fibroplasia and previous epididymo orchitis.[5-8] Another possible mechanism reported only recently by Adachi et al. is cholesterol embolism-associated STI.[8] However, the majority of cases have been reported as idiopathic.[5]
In this case the patient was initially treated for acute epididymitis with antibiotics by his general physician. Although it may be possible that this patient developed an STI secondary to epididymitis, our patient had no clinical, laboratory, or echographical signs for this condition. In addition, there was no history of systemic disease or genital trauma. In this  case therefore, the STI could be considered idiopathic.
The majority of cases of STI reported in the literature underwent a (partial) orchiectomy because of the suspicion of malignancy or the need for histology for definitive diagnosis.[5] However, a conservative approach to STI is possible and safe if the diagnosis is reached with a high degree of certainty.[9,10] In this patient we had not reached a high degree of certainty. The age of the patient, the acute onset of pain and negative tumor markers were suggestive of a non malignant condition. However, acute scrotal symptoms may occur in patients with testicular tumors.[11] On the other hand, weight loss of 11 kilograms and the firm mass palpable within the parenchyma were more suggestive of a possible malignancy.
Although ultrasound is the first choice diagnostic tool in evaluating scrotal pain, distinguishing the hypoechogenic lesion from a testicular tumor on ultrasound was difficult in this case. This difficulty in distinguishing between testicular infarction and testicular tumor, especially in small sized lesions, is also reported by other authors.[4,12] STI in adults usually is wedge shaped on ultrasound as is demonstrated by the study of Fernández-Pérez et al.[13]  In our patient, the lesion was oval shaped. Several studies have also reported a round morphology. [5,6,13]
Color Doppler ultrasound showed an avascular central zone with several peri-lesional spots with low colour signals. However, on Color Doppler ultrasound it can be difficult to detect increased blood flow in tumors less than 2 cm in size.[14] In any case, the presence of hypervascularity is not specific for the diagnosis of malignancy, because of the frequent hypovascular pattern of testicular tumors.[15] Therefore, an intratesticular tumor could not be excluded in our patient despite the absence of blood flow in the center of the hypoechogenic lesion.
When a discrepancy between clinical and ultrasonographic findings exists, MRI with contrast may provide additional information over ultrasound, occasionally showing a well-circumscribed hypo-intense area with an enhanced rim, indicating an STI.[13] Nevertheless, definite exclusion of a testicular tumor is not always possible because MRI may demonstrate different findings for different stages of infarction, showing lesions that are lacking the typical features previously mentioned.[16] The use of contrast enhanced ultrasound in the diagnosis of STI seems promising but requires further investigation.[6]
When STI cannot be diagnosed based on the clinical picture and radiologic findings, histological examination is necessary. In most studies STI is histologically confirmed following radical or partial orchiectomy.[4,8,16,19,20] However, in certain cases sparing of the testis is desired and a less invasive way for obtaining a histological confirmation is needed. Perioperative frozen section examination has proven to be accurate for distinguishing benign from malignant lesions, allowing the testis to be spared. In addition, there is no evidence that oncological safety is impaired with this technique.[17] Several studies have showed the feasibility of this method of confirming histology.[1,12,21] In the case of nonpalpable or small hypoechogenic lesions, intraoperative ultrasound guided needle localization with microsurgical exploration has proven to be a safe and effective approach for microsurgical removal of the lesion and obtaining histology. This allows maximal preservation of the testicular parenchyma and its vasculature.[22,23] In this case frozen section examination was not performed, because the patient did not   want to take any risk of the tumor being missed on the frozen section. In addition, because of his age our patient was not troubled by the fact of having one testis for the rest of his life.
As previously mentioned, conservative management has been proven to be possible and safe in highly selected patients where a diagnosis of STI has been made with a high degree of certainty. According to the literature, patients clinically supected to have for STI, with negative tumor markers and Doppler ultrasound and contrast enhanced MRI indicating a STI, are suitable for conservative management with scrotal ultrasound follow-up. Several studies report the development of the ultrasound appearance of STI during follow up ranging from one day to five years.[6,7,9,10] In these studies, follow-up ultrasound demonstrated no progression, or a gradual reduction in size of the lesion, with reflectivity and vascularity essentially unchanged or diminished. This could differentiate STI from a testicular tumor.
In conclusion, differential diagnosis between STI and testicular maligancy remains difficult. STI is therefore mostly detected after (partial) orchiectomy. Some features may suggest this benign condition; a hypovascular wedge-shaped lesion, in patients with normal tumor markers. In highly selected patients,  diagnosis with a high degree of certainty allows conservative treatment with strict follow-up ultrasound. However, if the clinical picture and radiologic imaging are inconclusive or unclear, histologic confirmation cannot be avoided. In that case explorative surgery together with (microsurgical) frozen section examination should be performed, especially in patients in whom sparing of the testis is desired. In the future, further studies are needed to better diagnose this benign condition.

 

References
 
1) Kim HK, Goske MJ, Bove KE, Minovich E. Segmental testicular infarction in a young man simulating a testicular tumor. Pediatr Radiol. 2009;39:400–402
2) Fukuda S, Takahashi T, Kumori K, Takahashi Y, Yasuda K, Kasai T, Yamaguchi S. Idiopathic testicular infarction in a boy initially suspected to have acute epididymo-orchitis associated with mycoplasma infection and Henoch-Schönlein purpura. J Pediatr Urol. 2009;5:68-71
3) Barber TD, Al-Omar O, Poulik J, McLorie GA. Testicular infarction in a 12-year-old boy with Wegener’s granulomatosis. Urology 2006;67:846.e9-10
4) Ruibal M, Quintana JL, Fernández G, Zungri E. Segmental testicular infarction.
J Urol.2003;170:187-188.
5) Gianfrilli D, Isidori AM, Lenzi A. Segmental testicular ischaemia: presentation, management and follow-up. Int J Androl. 2009;32:524-31
6) Bertolotto M, Derchi LE, Sidhu PS, Serafini G, Valentino M, Grenier N, Cova MA. Acute segmental testicular infarction at contrast-enhanced ultrasound: early features and changes during follow-up. AJR Am J Roentgenol. 2011;196:834-41
7) Bilagi P, Sriprasad S, Clarke JL, Sellars ME, Muir GH, Sidhu PS. Clinical and ultrasound features of segmental testicular infarction: six-year experience from a single centre. Eur Urol. 2007;17:2810-2818
8) Adachi S, Tsutahara K, Kinoshita T, Hatano K, Kinouchi T, Kobayashi M, Inoue H, Takada T, Hara T, Yamaguchi S. Segmental testicular infarction due to cholesterol embolism: not the first case, but the first report. Pathol Int. 2008;58:745-8
9) Madaan S, Joniau S, Klockaerts K, DeWever L, Lerut E, Oyen R, Van Poppel H. Segmental testicular infarction: conservative management is feasible and safe. Eur Urol. 2008;53:441-445
10) Madaan S, Joniau S, Klockaerts K, DeWever L, Lerut E, Oyen R, Van Poppel H. Segmental testicular infarction. Conservative management is feasible and safe: part 2. Eur Urol. 2008;53:656-658
11) Guthrie JA, Fowler RC. Ultrasound diagnosis of testicular tumours presenting as epididymal disease. Clin Radiol. 1992;46:397-400
12) Calcagno C, Gastaldi F. Segmental testicular infarction following herniorrhaphy and varicocelectomy. Urol Int. 2007;79:273-275
13) Fernández-Pérez GC, Tardáguila FM, Velasco M, Rivas C, Dos Santos J, Cambronero J, Trinidad C, San Miguel P. Radiologic findings of segmental testicular infarction. AJR Am J Roentgenol. 2005;184:1587-1593
14) Venugopal S, Schoeman D, Damola A, Hamid B, Powell C. Acute scrotal pain with a twist. Ann R Coll Surg Engl. 2010;92:24-26
15) Horstman WG, Melson GL, Middleton WD, Andriole GL. Testicular tumors: findings with color Doppler US. Radiology 1992;185:733-737
16) Kodama K, Yotsuyanagi S, Fuse H, Hirano S, Kitagawa K, Masuda S. Magnetic resonance imaging to diagnose segmental testicular infarction. J Urol. 2000;163:910–911
17) Kirkham AP, Kumar P, Minhas S, Freeman AA, Ralph DJ, Muneer A, Allen C. Targeted testicular excision biopsy: when and how should we try to avoid radical orchiectomy? Clinic Radiol. 2009;64:1158-1165
18) Sharma SB, Gupta V. Segmental testicular infarction. Indian J Pediatr. 2005;72:81-82
19) Secil M, Kocyigit A, Aslan G, Kefi A, Ozdemir I, Tuna B, Yorukoglu K. Segmental testicular infarction as a complication of varicocelectomy: sonographic findings. J Clin Ultrasound. 2006;34:143-145
20) Flanagan JJ, Fowler RC. Testicular infarction mimicking tumour on scrotal ultrasound – a potential pitfall. Clin Radiol. 1995;50:49-50
21) Hidalgo J, Rodríguez A, Canalias J, Muntané MJ, Huerta MV, Carrasco N, Vesa J. Segmental testicular infarction vs testicular tumour: the usefulness of the excisional frozen biopsy. Arch Esp Urol. 2008;61:92-93
22) Hopps CV, Goldstein M. Ultrasound guided needle localization and microsurgical exploration for incidental nonpalpable testicular tumors. J Urol. 2002;168:1084-1087
23) Kravets FG, Cohen HL, Sheynkin Y, Sukkarieh T. Intraoperative sonographically guided needle localization of nonpalpable testicular tumors. AJM Am J Roentgenol. 2006;186:141-143

 

Date added to bjui.org: 18/06/2012

DOI: 10.1002/BJUIw-2012-003-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 and Secondary Mesothelioma of the Tunica Vaginalis: a comparative case study

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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.
3. De Lima GR, de Oliveira VP, Reis PH, Pinheiro FG, Lima MV, Gonzaga-Silva LF. A rare case of Malignant Hydrocele in a young patient. J Paediatr Urol.  2009 Jun; 5(3):243-5; Epub 2008 Dec 12.
4. Cancer Research UK. Mesothelioma: Risks and Causes. https://cancerhelp.cancerresearchuk.org/type/mesothelioma/about/mesothelioma-risks-and-causes
5. Abe K, Kato N, Miki K, Nimura S, Suzuki M, Kiyota H, Onodera S, Oishi Y. Malignant mesothelioma of testicular tunica vaginalis. International Journal of Urology 2002. 9:602-603.
6. García de Jalón A, Gil P, Azúa-Romeo J, Borque A, Sancho C, Rioja L.A; Malignant mesothelioma of the tunica vaginalis: Report of a case without risk factors and review of the literature. International Urology and Nephrology 2003. 35(1):59-62
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

Report of the management of bilateral, plurifocal sertoli cell adenomas

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A twenty two year old male was referred a urology outpatient clinic for the evaluation of persistent scrotal discomfort following a culture proven E. Coli urinary tract infection(UTI). 

Authors: Moran, Diarmaid; Thomas, Arun; Grainger, Ronald
 
Corresponding Author: Diarmaid Moran, St James Hospital, Urology, Dublin, Ireland.   Email: [email protected]

Case Report
 
A twenty two year old male was referred to our urology outpatient clinic for the evaluation of persistent scrotal discomfort following a culture proven E. Coli urinary tract infection(UTI). His initial UTI had been successfully treated by his General Practitioner with oral ciprofloxacin.  The patient had no significant medical or psycho-social history, was sexually inactive and had no family history of note. Other than vague scrotal discomfort that was persistent for six weeks he had no genito-urinary symptoms.
General and abdominal examination were normal. Specifically his height and weight were within the normal range, he had no gynaecomastia, had normally distributed body and pubic hair and had no excessive skin freckling around his head area.  Examination of his scrotum revealed small testes bilaterally. The right testicle had a small, palpable and tender nodule in the interpolar region. Examination of the left testicle was normal.
A  testicular ultrasound demonstrated multiple hypoechoic lesions within both testes (Fig. 1).
 

Figure 1. A testicular ultrasound demonstrated multiple hypoechoic lesions within both testes

 

 

The largest lesion (palpable) measured 1 x 0.7cm. There was no associated increase in vascularity and both epididymides were normal in appearance with no ultrasonic features of epididymo-orchitis. The radiological findings were reported a consistent with lymphoma or less likely multifocal seminoma.
A follow-up CT scan of his neck, thorax, abdomen and pelvis demonstrated no evidence of para-aortic lymphadenopathy or metastatic disease. Testicular tumour markers were all within the normal range. The case was discussed both pre and post operatively at our institution’s multi-disciplinary team meeting.
The patient underwent right testicular exploration via an inguinal approach. Intra-operative examination of the right testis revealed a small testis with a 10mm interpolar nodule corresponding to clinical and radiological findings. The nodule was locally excised and sent for frozen section evaluation.  Grossly, this 10 x 10 x 8mm nodule had a glistening outer surface and when sectioned had a smooth, cream surface.  Histological examination showed a benign sertoli cell hamartoma (adenoma) with no evidence of intratubular germ cell neoplasia. Two further ultrasound guided biopsies of the same testis were performed. Repeated frozen section analysis of these separate lesions demonstated atrophic seminiferous tubules with focally prominent Leydig cells, pathologically typical of a cryptorchid testis.

 

Fig.2. Sertoli cell hamartoma stained with hematoxylin and eosin stain at 20x (A), 100x (B), 200x (C) and 400x (D)and magnification

 


 
Both testes were preserved and the patient made an uncomplicated post operative recovery. At a six month review,  all genitourinary symtoms had resolved, with no evidence of testicular atrophy or endocrine dysfunction. Serum testosterone was normal. Follow-up clinical and ultrasonic examination findings of both testes  at 6 weeks and 6 months remained unchanged. The patient performs regular self examination and attends for annual review.

 

Discussion
 
Testicular sertoli tumours, first described by Teilum in 1944, account for 1% of all testicular neoplasms and 17% of non germ cell tumours. The majority of sertoli tumours are benign but 10% to 22% have malignant potential with less than 50 cases worldwide [1,2].  Five histological variants are recognised; classic sertoli tumour, large cell calcifying,  sclerosing, heterongenous and not otherwise specified (NOS). While sertoli cell tumours were initially reported to present most commonly in the first decade of life,  many of these early descriptions occurred before the recognition of testicular juvenile granulosa tumours in 1979. The dramatic reduction in the reporting of sertoli cell tumours in children since then suggests that many of these early reports may have been incorrectly classified [3]. Sertoli cell tumours may still occur in children, but presentation in the 3rd to 5th decade is more typical.  Sertoli cell tumours occur in both testes equally, usually presenting as a gradually enlarging testicular mass or as an incidental finding on ultrasound. While hormonal abnormalities are infrequent, gynaecomastia is sometimes seen (5% in benign / 12% in malignant tumours) [4]. Only 11 cases of bilateral sertoli cell tumours are reported in the literature[5-15]. One case of bilateral, plurifocal sertoli cell tumours has been documented previously, occuring in a single cryptorchid testes of a 14 year old girl with testicular feminisation syndrome [7]. Other anecdotal reported conditions associated with sertoli cell tumours include cryptorchidism, Peutz-Jaeger syndrome, the Carney complex and the aquired immunodeficiency syndrome [16]. Systemic clinical features of these associated conditions or systemic effects from hormonally active sex cord-stromal tumours may be the presenting feature which prompts the patient (or their parents) to seek medical attention. In this case, apart from the testicular abnormalities described, our patient had a normal clinical examination at presentation with no other systemic features to suggest the presence of an associated syndrome.
As with most testicular tumours, the standard treatment of unilateral sex chord scrotal tumours is radical inguinal orchidectomy. It permits a definitive pathologic diagnosis and provides local tumour treatment. However the European Association of Urology clinical guidelines suggest that testis-sparing surgery may be considered as an alternative treatment modality in certain circumstances provided surgical rules and principles are respected [17]. This form of treatment should be considered in; a tumour in a solitary testes with normal pre-operative testosterone levels, synchronous bilateral testicular tumours, metachronous contralateral tumours, tumour volume of less than 2 cm or less than 30% of the testicular volume, and infertile / monorchid patients. Secondary orchidectomy can always be performed if the final pathology reveals a non-stromal (e.g. germ cell) tumour.
Our case showed no pre or intra-operative evidence of malignancy. Therefore we elected to perform testis sparing surgery. The rationale for pursuing this clinical management course included consideration of the patient’s age (22 years) and the detrimental impact (both physical and psychological) of performing bilateral radical orchidectomies on a patient in this age group. By preserving both testicles, we have maximised his future fertility potential and have preserved endocrine, exocrine and sexual functions of the testis.
Due to the rarity of the condition and the lack of follow-up in most reported cases, the EAU guidelines on testicular cancer [17] does not make specific recommendations regarding the appropriate follow-up for patients with sertoli-cell tumours. However these guidelines do suggest that an individualised follow-up plan, tailored to the clinical needs of the patient, be implemented. Our patient has had a six month surveillance scrotal ultrasound which has demonstrated no change in comparison to his initial examination and will continue to be reviewed at our urology outpatient department on an annual basis.

 

Conclusion
 
Management of benign but potentially malignant bilateral testicular tumours in a young adult male is challenging. A balance between preserving testicular function and optimizing oncological control has to be considered. Without evidence of malignancy, testicular preserving surgery should be considered. The patient needs to be fully informed regarding the nature of the condition and the management options available. An individualised post intervention surveillance strategy needs to implemented to provide optimal patient care.

 

References
1. Krege S et al. European consensus conference on diagnosis and treatment of germ cell cancer: a report of the second meeting of the European Germ Cell Cancer Consensus Group (EGCCCG): part II. Eur Urol 2008 Mar; 53 (3):497-513.
2. Souchon R, Schmoll HJ, Krege S, for the German Testicular Cancer Study Group (eds). Qualitätsicherung in der Onkologie. 1st edn. München-Bern-Wien-New York- Zuckschwerdt, 2002. German Cancer Society: Evidence-based guideline for the assessment and treatment of testicular tumours.
3. Young RH, Rosenberg AE, Clement PB. Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases. Mod Pathol. 1997; 10 (12):1228-32.
4. McGlynn KA, Devesa SS, Sigurdson AJ, Brown LM, Tsao L, Tarone RE. Trends in the incidence of testicular germ cell tumours in the United States. Cancer 2003; 97 (1): 63-70.
5. Brown B, Ram A, Clayton P, Humphrey G. J Pediatr Surg. 2007; 42 (9): 13-5.Conservative management of bilateral Sertoli cell tumors of the testicle in association with the Carney complex: a case report.
6. Baksu A, Kabukcuoglu F, Baksu B, Goker N. Sertoli cell adenoma and serous cyst in a patient with androgen insensitivity syndrome. Eur J Obstet Gynecol Reprod Biol. 2004 10; 114 (1): 104-7.
7. Chatelain D, Ricard J, Ghighi C, Colombat M, Leclercq F, Cordonnier C, Pouzac M, Sevestre H, Gontier MF. Plurifocal testicular hamartomas and testicular feminization syndrome. Ann Pathol. 2000; 20 (6): 605-8.
8. Drevelengas A, Kalaitzoglou I, Destouni E, Skordalaki A, Dimitriadis A. Bilateral Sertoli cell tumor of the testis: MRI and sonographic appearance. Eur Radiol. 1999; 9 (9): 1934.
9. White MD, Loughlin MW, Kallakury BV, Ross JS, Mandell J. Bilateral large cell calcifying Sertoli cell tumor of the testis in a 7-year-old boy. J Urol. 1997 158 (4): 1547-8.
10. Noszian IM, Balon R, Eitelberger FG, Schmid N. Bilateral testicular large-cell calcifying sertoli cell tumor and recurrent cardiac myxoma in a patient with Carney’s complex. Pediatr Radiol. 1995; 25 Suppl. 1: S236-7.
11. Dreyer L, Jacyk WK, du Plessis DJ. Bilateral large-cell calcifying Sertoli cell tumor of the testes with Peutz-Jeghers syndrome: a case report. Pediatr Dermatol. 1994; 11 (4): 335-7.
12. Niewenhuis JC, Wolf MC, Kass EJ. Bilateral asynchronous Sertoli cell tumor in a boy with the Peutz-Jeghers syndrome. J Urol. 1994; 152 (4):1246-8.
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Date added to bjui.org: 16/08/2011 


DOI: 10.1002/BJUIw-2011-047-web

 

Polyorchidism in children

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We report a case of a very rare right-sided polyorchidism in a child, review the reported pediatric cases and discuss the management of this anomaly. 

 

Authors: Dushi, Gezim; Ramseyer, Pascal; Meyrat, Blaise; Frey, Peter. CHUV, Pediatric Urology, Lausanne, Switzerland.
Corresponding Author: Peter Frey, CHUV, Pediatric Urology, Lausanne, Switzerland.  Email: [email protected]

Introduction
Polyorchidism, a rare anomaly, signifies presence of more than two testes. Since the first case, found on a routine autopsy by Blasius in 1670 [1], further 191 cases in a mixed adult and pediatric population were reported until recently [2]. Most of the cases presented were left sided triorchidism.
After literature review, we could not find any specific analysis of polyorchidism in the pediatric population. We therefore report a case of a very rare right-sided polyorchidism in a child, review the reported pediatric cases and discuss the management of this anomaly.

 

Case report
A 13 year-old-boy presented at the out-patient clinic for investigation of a painless soft tissue mass in the right scrotum. The ultrasound (fig. 1) revealed two normal homogenous structures, being testes, in the right scrotum. The cranial one was bigger than the caudal and measured 1.8x2x2.7cm compared to the caudal, measuring 1.3×1.4×1.6cm. An epididymis attached to each testis was seen. There was only one testis on the left side, which was normal on ultrasound. MRI (fig.2) showed that the epididymi of each right-sided testis were attached to each other and only one vas deferens was seen. This presentation of polyorchidism is classified according to Leung [3] as type 3, to Singer [4] as type 1, to Thum [5] as type II and to Bergholz [6] as type A3.
The tumor markers alpha-fetoprotein, prostate-specific antigen and beta-human chorionic gonadotropin were negative. There was no surgery performed. Patients were followed-up, every 6 months, clinical examination was uneventful and annual ultrasound was normal 3 years after the diagnosis of polyorchidism. Clinical and ultrasound examination will be further performed in the future.

 

Figure 1. Ultrasound: Presence of two homogenous testes. The caudal testis has its own epidydimis (circle or arrow)

 

 

Figure 2. MRI: Sagittal view T2-weighted. Double testes on the right, sharing the same vas deferens. (arrow)

 

 

Literature review
 
Methods
Using the combinations of key words “polyorchidism”,” triorchidism”, “supernumerary testes” “boy”, “child” we searched the electronic databases MEDLINE® and EMBASE®. Histologically proven cases of polyorchidism or cases diagnosed by imaging techniques were included in our study, the latest entry being in February 2011.

 

Results
47 cases of polyorchidism were reported in children aged from 1 month to 16 years ( mean 6.8 years). Only one case of bilateral testicular duplication was reported.
There is a predominant 83% left-side presentation. 21 (42.5%) were discovered during surgical intervention for testicular maldescent, hernia in three (6.5%), testicular torsion in two (4%), pain in two (4%) cases, hydrocele in one (2%) and scrotal lymphangioma in one (2%) case. In 18 (38%) children the polyorchidism was found during the evaluation of a palpable scrotal mass from which 10 (55%) were operated. Presence of a seminoma and a teratoma was reported in two respective cases (4%).

 

Discussion
Polyorchidism is a rare congenital malformation. The left-sided predominance encountered in adults  (91.65% of cases) [2] was confirmed in the pediatric population where it was found in 83% of cases. Earlier reports of abnormalities associated with polyorchidism showed an association of 40% with maldescended testis, 30% with hernia, 15% with torsion, 9% with hydrocele and 6% with tumors in a mixed adult and pediatric population [7]. In a more recent meta-analysis by Bergholz [2] the reported percentages were almost equal.
In the pediatric group, however, in the literature this was shown to be true only in the case of maldescended testis (42.5%) and tumors (4%). In all other associated anomalies the percentages were much lower. In children, scrotal pain leading to the discovery of polyorchidism was low (4%) and comparable to the one reported in the mixed population [2].
The reported presence of painless scrotal mass in children was high (38%), which is in accordance with earlier reported results [3] but in contrary to the recent meta-analysis [2] where only 16% of the patients presented with this feature.
With the advances in imaging techniques (US and MRI) the former practice of removing the supernumerary testes has changed. The conservative surveillance of polyorchidism in cases with a normal radiological appearance and negative tumor-markers has started to be accepted, especially in the pediatric population [8-13].
Despite this fact, we found that 55% of cases of polyorchidism in children, diagnosed by ultrasound for a painless scrotal mass, were operated on.
There are mainly two possible classifications of polyorchidism in the literature: the etiological [3,5] and, since the etiology is not clearly understood, the anatomical [4,6] classification was developed.
We propose a rather simple pragmatic approach in children with the aim to preserve their reproductive capacity and to avoid potential operation-related complications.
This management strategy is similar to the one published by Khedis et al [14], however,  it has been simplified and in particular suggests an ultrasound to be performed on a regular and compulsory basis. Also it differs from the strategy suggested by Repetto et al [8], as we do not suggest performing repeated MRI.
In cases of  polyorchidism recognised by the presence of a scrotal mass, and clearly identified by imaging techniques and with negative tumor markers, we propose conservative treatment, performing clinical and ultrasound examination at 6 monthly and yearly intervals respectively.
In cases of  polyorchidism discovered during surgical intervention, we advocate orchidectomy if the supernumerary testis is atrophic, separated from the normal testis, or without connection to the vas deferens. These were formerly classified according to Leung as type 1, to Singer as type 2, to Thum as type I and to Bergholz as type B1 and 2.
In all the other cases, formerly classified according to Leung type 2,3 and 4, to Singer as type 1; to Thum as type II and III, and to Bergholz as type A1,2 and 3 we propose conservative management and clinical follow-up as described above.
Conclusions: The optimal management of polyorchidism is still a matter of debate. After literature review in children we propose whenever possible conservative management, however, these patients should be very closely followed up.

 

References
 
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Date added to bjui.org: 02/08/2011 


DOI: 10.1002/BJUIw-2011-043-web
 

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