ABSTRACT

Primary non-Hodgkin lymphoma of the testis is a rare disease and accounts for 9% of testicular tumors. Primary testicular Diffuse large B-cell lymphoma (DLBCL) has a poor prognosis despite localized presentation and doxorubicin-based chemotherapy. The differential diagnosis of primary testicular DLBCL includes seminoma, chronic orchitis, plasma cell neoplasm, follicular lymphoma, extramedullary myeloid tumor, peripheral T-cell lymphoma, extranodal Natural killer/T-cell lymphoma, blastoid or pleomorphic variants of mantle cell lymphoma, Burkitt lymphoma, acute lymphoblastic leukemia, and secondary testicular involvement by systemic lymphomas. Most of the testicular involvement occurs concurrently with bone marrow (BM) involvement. The BM relapse and early relapse in acute lymphoblastic leukemia, as well as T-cell phenotype of leukemic blasts, are associated with worse prognosis than the isolated extramedullary or late relapse. Extramedullary myeloid tumor is a tumor mass composed of immature myeloid cells, which occur outside the BM.