Blackwell Science, LtdOxford, UKIJUInternational Journal of Urology1442-20422004 Blackwell Publishing Asia Pty LtdDecember 2004122223224Case ReportTesticular NK/T-cell lymphomaC Ballereau et al.
International Journal of Urology (2005) 12, 223–224
Case Report
Testicular natural killer T-cell lymphoma CHARLES BALLEREAU,1 XAVIER LEROY,2 FRANCK MORSCHHAUSER,3 JEAN-CHRISTOPHE FANTONI,1 LAURENT LEMAITRE,4 ARNAULD VILLERS1 AND JACQUES BISERTE1 Departments of 1Urology, 2Pathology, 3Hematology and 4Radiology, Center Hospitalier Régional Universitaire (CHRU) de Lille, Lille, France Abstract
Primary testicular lymphomas account for less than 5% of all testicular neoplasms. Testicular natural killer (NK)/T-cell lymphomas are exceptional and have a very poor prognosis. We report the case of a 30-year-old French Caucasian man with testicular NK/T-cell lymphoma of the following immunophenotype: CD2+, CD3e+ and CD56+. Despite intensive chemotherapy, the disease progressed rapidly, with death occurring 2 months after diagnosis.
Key words
killer cells, lymphoma, T-cell, testicular neoplasms.
Introduction Primary testicular lymphomas account for less than 5% of all testicular neoplasms. The majority are diffuse large B-cell non-Hodgkin lymphomas. Their prognosis is poor.1,2 A very rare subtype is the natural killer (NK)/ T-cell lymphoma with the immunophenotype CD2+, CD3e+ and CD56+. We describe a case of testicular NK/T-cell lymphoma.
Case report A 30-year-old white (French Caucasian) man presented with a 3-week history of bilateral scrotal swelling and fever. He received antibiotics (trimethoprim and sulfamethoxazole) for suspected orchitis infection and was admitted to hospital because of pancytopenia and loss of weight. Physical examination revealed a bilateral tumor of the testis and skin vasculitis. The results of a bone marrow puncture examination were normal. Ultrasonography revealed bilateral testicular enlargement (44 cc right testis, 29 cc left testis) and multifocal, intensely hypoechoic areas, which showed enhanced Correspondence: Charles Ballereau MD, Department of Urology, Center Hospitalier Régional Universitaire (CHRU) de Lille, 59037 Lille, France. Email:
[email protected] Received 5 February 2004; accepted 27 July 2004.
flow velocity on Color Doppler sonography. No lymph node enlargement was found on abdominal computerized tomography (CT). The testis was the only site of disease on staging. A right radical orchiectomy was performed. The testis consisted of a large firm white tumor mass (6.4 ¥ 4 ¥ 3 cm) composed of sheets of medium-tolarge lymphoid cells infiltrating the seminiferous tubules. The cytoplasm of these cells was abundant; the cells were multinuclear; the nuclei were round and irregular (Fig. 1). No plasma cells were seen. Numerous mitotic figures, rare areas of tumor necrosis and angioinvasion were observed. Immunohistochemistry studies on paraffin-embedded tissue showed positivity of the tumor cells for antibodies to CD3e, CD56 (Fig. 2) and CD43. No staining was observed with antibodies to CD20, CD79a or CD30. On frozen sections, CD3 and CD19 were negative. In situ hybridization showed >50% positivity for Epstein–Barr virus-encoding RNA. The results of gene rearrangement studies for the gamma chain of the T-cell receptor were negative. The diagnosis was primary testicular NK/T-cell lymphoma. The patient received a complete course of intensive chemotherapy (doxorubicine, cyclophosphamide, vindesine, bleomycine, methotrexate), but the disease progressed, with enlargement of the left testicle and encephalitis. Chemotherapy with cytarabine and etoposide was proposed, but the patient developed acute multiorgan failure and died of disease 2 months after the diagnosis.
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Fig. 1 Testicular natural killer/T-cell lymphoma: Histology (¥25).
prisingly, our patient was of Caucasian origin. This is exceptional, because reported cases of extra-nasal, like nasal, NK/T-cell lymphomas seem to have concerned individuals of Asian, Mexican or South American descent.4 The characteristics of the NK/T-cell lymphoma in our patient were typical: predominant extranodal presentation, admixture of small-to-large lymphoid cells, angiocentric growth, and large granular lymphocyte morphology. The most common immunophenotype is: CD2+, CD3–, CD16+/–, CD56+, CD57–.5 NK/T-cell lymphomas are strongly associated with Epstein–Barr virus infection.6 They are highly aggressive with early skin, aerodigestive tract or soft tissue involvement and often severe hemophagocytic syndrome; they have a very poor prognosis. Despite intensive chemotherapy, disease progressed rapidly in our patient leading to death within 2 months of diagnosis.
Acknowledgments We thank Claude Preudhomme for help in reading the cytology slides.
References 1 2
3
Fig. 2 Testicular natural killer/T-cell lymphoma: Immunostaining for CD56 (¥400).
4
Discussion
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The most common NK/T-cell lymphomas are nasal or nasal-type. Extra-nasal NK/T-cell lymphomas, such as the testicular lymphoma detected in our patient, are rare. Only nine cases of testicular NK/T-cell lymphomas have been reported so far.3 The tumor is often bilateral. Sur-
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Chan JK, Tsang WY, Lau WH et al. Aggressive T/ natural killer cell lymphoma presenting as testicular tumor. Cancer 1996; 77: 198–205. Totonchi KF, Engel G, Weisenberg E et al. Testicular natural killer/T-cell lymphoma, nasal type, of true natural killer-cell origin. Arch. Pathol. Lab. Med. 2002; 126: 1527–9. Kim YB, Chang SK, Yang WI et al. Primary NK/T-cell lymphoma of the testis. A case report and review of the literature. Acta Haematol. 2003; 109: 95–100. Kluin PM, Feller A, Gaulard P et al. Peripheral T/NKcell lymphoma: a report of the IXth Workshop of the European Association for Haematopathology. Histopathology 2001; 38: 250–70. Chan JK, Sin VC, Wong KF et al. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 1997; 89: 4501–13. Ko YH, Cho EY, Kim JE et al. NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status. Histopathology 2004; 44: 480–9.
