Unusual coexistence of plasmablastic lymphoma and mature cystic teratoma of the ovary

Background: Plasmablastic lymphoma (PBL) is relatively new clinical entity described as a distinct subtype of diffuse large B-cell lymphoma (DLBCL), characterized by its aggressive nature and proliferation of large neoplastic cells resembling immunoblasts to cells with more obvious plasmacytic differentiation. Principals/Methodology: To describe an unexpected finding of PBL associated with mature cystic teratoma of the ovary in the young immunocompetent women. Results: A 19-vear old woman was admitted to the hospital with generalized lymphadenopathy, pelvic tumour mass measuring 35x30 cm and with 4cm lump in her right breast. The patient underwent right salpingo-oophorectomy, lymphadenectomy, removal of omentum, splenectomy and right breast lumpadenectomy. At macroscopic examination the right ovary was replaced by thick-walled multilocular cystic tumor. On incision, cysts were filled with thick, greasy sebaceous material and hair while within cysts walls there were several solid, greyish nodules. Histological examination revealed a mature cystic teratoma while solid nodules were malignant non-Hodgkin lymphoma (NHL). Tumour tissue from the right breast, spleen and lymph nodes, all had the same histological NHL morphology. After extensive immunostaining, a diagnosis of PBL was made. In the past year the patient was treated with IV cycles of EPOCH chemotherapy, I cycle of salvage DHAP chemotherapy and III cycles of CODOX-M/IVAC alternating chemotherapy but without any significant regression of the disease, as shown by the most recent PET-CT scan. Conclusion: PBL remains a diagnostic challenge given its peculiar morphology and immunohistochemical profile which is similar to anaplastic or plasmablastic plasma cell myeloma (PCM). Furthermore, PBL represents therapeutic challenge, with very aggressive clinical course and with majority of the patients dying in the first year after diagnosis.