Primary ovarian Burkitt lymphoma: report of a case and review of literature

We report a case of primary ovarian Burkitt lymphoma that occurred in a 25-year-old woman. The patient complained of a mass in the right ovary discerned by physical examination 2 months prior. Ultrasound examination indicated that the right ovary was enlarged and abundant blood flow signals were observed. Right salpingo-oophorectomy was subsequently performed. Histology was characterized by diffuse sheets of monotonous medium-sized lymphoid cells with plentiful mitotic figures and apoptosis. Numerous tingible-body macrophages were found in the ovarian tissue, presenting a starry sky pattern. The tumor cells expressed CD20, CD10, BCL6, and MYC in the absence of BCL2. Ki-67 proliferative index was very high with a proliferation rate of near 100%. MYC (8q24) rearrangement was detected by fluorescence in situ hybridization (FISH) with no BCL2 (18q21) and BCL6 (3q37) gene rearrangements. Cumulative evidence established primary ovarian Burkitt lymphoma as the final histopathologic diagnosis with clinical stage I (FIGO). The patient received HyperCVAD chemotherapy after surgery and remained complete response (CR) for 18 months. We aim to provide insight into the future treatment of this rare but lethal disease.