Tag Archive for: primary prostatic lymphoma

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

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.

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).

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

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

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

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

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

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

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].

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.

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.