Unusual primary presentations of mantle cell lymphoma in the urinary tract and testes.
e18503 Background: The initial clinical presentation of Mantle Cell Lymphoma is protean, ranging from single sites of indolent disease followed for years without therapy to aggressive disseminated disease that requires treatment within weeks of diagnosis. While many cases of Mantle Cell Lymphoma present with disseminated disease, other patients may present with symptoms related to a primary extranodal site of disease. The majority of these primary extranodal cases are diagnosed in the gastrointestinal tract, other cases have been noted in the skin, conjunctiva, testes and male genitourinary tract. Methods: Database review of 204 patients diagnosed with Mantle Cell Lymphoma at Moffitt Cancer Center between May 1992 and December 2010. Results: Three cases of Mantle Cell Lymphoma presented with symptoms related to primary extranodal sites in the urinary tract and male genital organs. Two cases of testicular involvement presented with gradual enlargement of the testes. In patient 1, the orchiectomy specimen harbored both seminoma and mantle cell lymphoma. Low level bone marrow involvement by mantle cell lymphoma was present and in the presence of indolent features he was treated for his primary seminoma and followed expectantly for mantle cell lymphoma. In patient 2, orchiectomy revealed a pleomorphic variant of mantle cell lymphoma with no other sites of disease on staging workup. He was treated with 6 cycles of R-CHOP and prophylactic IT methotrexate with a complete remission. A third patient presented with obstructive urinary symptoms and was found to have mantle cell lymphoma of the prostate. Additional staging revealed diffuse lymphadenopathy, colon and bone marrow involvement. A complete remission was obtained with 6 cycles of R-CHOP. None of these three patients was found to have evidence of CNS involvement at diagnosis or in followup with only the second patient receiving intrathecal prophylaxis. Conclusions: Mantle Cell Lymphoma may present with unusual extranodal involvement at diagnosis including sites and symptoms related to the genitourinary tract and testes. Diagnostic lumbar puncture and intrathecal prophylaxis should be considered in these patients.