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Primary prostatic intravascular large B cell lymphoma

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
 
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Date added to bjui.org: 10/03/2012 

DOI: 10.1002/BJUIw-2011-102-web

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