BENIGN HEMATOLOGIC NEOPLASM ASSOCIATED WITH MEDIASTINAL MATURE TERATOMA IN A PATIENT WITH KLINEFELTERS-SYNDROME - A CASE-REPORT

An 18-year-old male with Klinefelter's syndrome presented with a matur e teratoma of the mediastinum. He was treated with debulking surgery a nd brief cisplatin-based chemotherapy. Ten years later, he presented w ith paraplegia and was found to have an isolated epidural mass. The ma ss was composed of mature histiocytoid cells reminiscent of those seen in malignant histiocytosis. There was no evidence of medullary dissem ination and there was no evidence of recurrence of the germ cell tumor . He received local radiation therapy and a single course of leukemic induction. He remains well 4 years later. This case represents a local ized variant of the diffuse histiocytic malignancies associated with m alignant mediastinal germ cell tumors. (C) 1994 Wiley-Liss, Inc.