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.
Zaloudek
C
C
Department of Pathology, University of California, San Francisco 94143, USA.
Miller
T R
TR
Stern
J L
JL
D000328:Adult; D003893:Desmin / Q000032:analysis; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007633:Keratins / Q000032:analysis; D009362:Neoplasm Metastasis; D010051:Ovarian Neoplasms / Q000473:pathology* / Q000628:therapy; D011247:Pregnancy
D003893:Desmin; D007633:Keratins