Aggressive nasal-type natural killer/T-cell lymphoma associated with Epstein Barr virus presenting as testicular tumor.

Background: Testicular lymphoma is a lethal disease with a median survival of approximately 12 to 24 months. Nasal-type natural killer/T-cell lymphoma of the testis is exceptional whether as a primary or secondary tumor.

Aim: The authors report on the comprehensive histopathologic, immunohistochemical and molecular analysis of a case of primary testicular nasal type NK/T cell lymphoma and review the features of previously reported cases.

Observation: We report a case of primary nasal-type natural killer/T-cell lymphoma of testis in a 28-year-old male. The histopathological examination of the surgical specimen, showed a large lymphoma cells with angioinvasion expressing CD3 (cytoplasmic), CD2, CD8, CD43, CD45, CD45Ro, CD56, T-cell intracellular antigen-1, perforine, Mib1 and granzyme. In situ hybridation for Epstein-Barr-virus- encoded mRNA was positive. Polymerase chain reaction study of formalin-fixed tissue showed lack of T-cell receptor gene rearrangements. The initial stage was I (EA) of Ann Arbor. This lymphoma was refractory to chemotherapy. The patient developed lymph node metastases in the out iliac and in the subclavicular region two months later. He died of disease after eight months.

Conclusion: This study confirms that testicular NK/T-cell lymphoma deserves to be distinguished from the other testicular lymphomas. In fact, this lymphoma tends to occur at young age, to disseminate early, to have an aggressive course, and is strongly associated with EBV.