Desmoplastic small cell tumor of the ovary: a unique polyphenotypic tumor with an unfavorable prognosis.

Desmoplastic small cell tumor (DSCT) is a recently described intraabdominal neoplasm of uncertain histogenesis that occurs predominantly in boys and young men. We report a case in a young woman that presented clinically as bilateral ovarian tumors with extensive pelvic and abdominal dissemination. The patient had cytoreductive surgery followed by combination chemotherapy. She had a complete clinical remission, but tumor recurrence was detected 1 year after diagnosis and the patient died 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 diagnosis of small cell tumors of the ovary.