A Case of EBER Positive Angioimmunoblastic T-cell Lymphoma Combined with Myxofibrosarcoma of the Pleura.

A 76-year-man presented with generalized lymphadenopathy. Lymph node biopsy led to the diagnosis of Epstein-Barr virus-encoded small RNA in situ hybridization (EBER)-positive angioimmunoblastic T-cell lymphoma (AITL). He was initiated on treatment with oral prednisolone (PSL) at the dose of 50 mg/day; however, he was diagnosed as having right pleural effusion. He was started on treatment with cyclophosphamide, doxorubicin, vincristine and PSL (CHOP therapy). However, the right pleural effusion increased in size, and thoracentesis was performed. The aspirated pleural fluid was bloody, and since only a very small number of atypical cells were found, no definitive diagnosis could be made. CT revealed multiple nodular lesions in the pleura, and thoracoscopy was performed, which revealed jelly-like white lesions in the right parietal pleura. Biopsy raised the suspicion of undifferentiated pleomorphic sarcoma (UPS). Treatment with carboplatin and pemetrexed was started, but his respiratory symptoms worsened and he died. Autopsy revealed evidence of complete remission of AITL and myxofibrosarcoma (MFS) of the pleura. This is the first reported case of AITL combined with MFS.