DESMOPLASTIC SMALL-CELL TUMOR OF THE OVARY - A UNIQUE POLYPHENOTYPIC TUMOR WITH AN UNFAVORABLE PROGNOSIS

Desmoplastic small cell tumor (DSCT) is a recently described intraabdo minal neoplasm of uncertain histogenesis that occurs predominantly in boys and young men. We report a case in a young woman that presented c linically as bilateral ovarian tumors with extensive pelvic and abdomi nal dissemination. The patient had cytoreductive surgery followed by c ombination chemotherapy. She had a complete clinical remission, but tu mor recurrence was detected 1 year after diagnosis and the patient die d of her disease 18 months after presentation. DSCT has a distinctive histologic appearance and a unique immunophenotype. It can present as an ovarian neoplasm and must be considered in the differential diagnos is of small cell tumors of the ovary.