Aggressive T/Natural killer cell lymphoma presenting as testicular tumor

• Published in: Cancer 1996-03, Vol.77 (6), p.1198-1205
• Main Authors: Chan, John K. C., Tsang, William Y. W., Lau, Wai‐Hon, Cheung, Michael M. C., Ng, Wing‐Fung, Yuen, Wai‐Cheung, Ng, Chi‐Sing
• Format: Article
• Language: English
• Subjects: Aged; Biological and medical sciences; CD2 Antigens - analysis; CD3 Complex - analysis; CD56; CD56 Antigen - analysis; Gynecology. Andrology. Obstetrics; Hematologic and hematopoietic diseases; Herpesvirus 4, Human - isolation & purification; Humans; Immunohistochemistry; In Situ Hybridization; Killer Cells, Natural - immunology; Killer Cells, Natural - pathology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, T-Cell - diagnosis; Lymphoma, T-Cell - immunology; Lymphoma, T-Cell - pathology; Male; Male genital diseases; Medical sciences; Middle Aged; natural killer cell; neural cell adhesion molecule; non‐Hodgkin's lymphoma; T cell lymphoma; T/NK cell lymphoma; Testicular Neoplasms - diagnosis; Testicular Neoplasms - immunology; Testicular Neoplasms - pathology; testicular tumor; Tumors
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/(SICI)1097-0142(19960315)77:6<1198::AID-CNCR29>3.0.CO;2-W

BACKGROUND Nonnasal CD56+ T/natural killer (NK) cell lymphomas with morphologic and immunogenetic features similar to those of the distinctive nasal T/NK cell lymphoma are uncommon and have been characterized only recently. They show predominantly extranodal presentation, high stage disease, a highly aggressive course, strong association with Epstein–Barr virus (EBV), and lack of T cell receptor gene rearrangement. Only one previously reported case had a testicular presentation, although the testis is not uncommonly involved during the course of disease in both nasal and nonnasal T/NK cell lymphomas. METHODS Three patients with T/NK cell lymphoma who presented initially with a testicular mass are reported. RESULTS The three patients underwent orchidectomy for testicular tumor. Histologically, the testes showed diffuse dense infiltration of medium‐sized or large lymphoma cells. Angiocentric growth and necrosis were prominent in two cases. The lymphoma had the following immunophenotype: CD2+ CD3ϵ+ CD56+ compatible with T/NK cell lymphoma; two lacked staining with Leu4 (CD3), and one had weak staining. With immunohistochemical preparations, it was noted that the rete testis stained consistently for CD56, and the Leydig cells and Sertoli cells showed patchy staining. The neoplastic cells harbored EBV, as demonstrated by in‐situ hybridization. Additional sites of disease were detected at the time of the diagnosis in one patient (nose) or appeared soon afterwards in all three patients (skin or gastrointestinal tract). All three patients died within 5 months. CONCLUSIONS This study confirms that testicular CD56+ T/NK cell lymphoma tends to disseminate early, pursues an aggressive course, and is strongly associated with EBV. CD56 recognizes the neural cell adhesion molecule (NCAM), which exhibits homophilic binding properties. The expression of CD56 in the normal testicular constituents can perhaps explain the tendency for T/NK cell lymphoma to localize in this organ. Cancer 1996;77:1198‐205.