title: A Unique Case of Extraovarian Sex-Cord Stromal Fibrosarcoma, With Subsequent Relapse of Differentiated Sex-Cord Tumor.
abstract: Primary fibrosarcoma arising from ovarian sex-cord stroma is a very rare neoplasm, with only a few reports in the literature. These tumors have been reported to express inhibin which allows their distinction from fibrosarcomas of soft tissue. Here, we report a case of a fibrosarcoma arising in the broad ligament. Despite being totally separate from the ovary, the tumor was diagnosed as sex-cord stromal type on the basis of inhibin expression. Furthermore, this patient suffered a recurrence of her tumor in the pelvis, which showed both the fibrosarcomatous, as well as other sex-cord elements, confirming the sex-cord stromal differentiation of the sarcoma. To our knowledge, this is the first case of a sex-cord stromal fibrosarcoma arising from an extraovarian site. Furthermore, this is also the first case of a recurrent fibrosarcoma, which showed redifferentiation of the tumor into other sex-cord components.
authors.affiliation: Departments of Histopathology (J.W., M.A.E.-B.) Oncology (P.P., P.S.) Gynecology (J.R.S.), Imperial College London Department of Histopathology (J.W., C.F.), Royal Marsden Hospital, London, UK.
authors.forename: Jayson
authors.initials: J
authors.lastname: Wang
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Papanastasopoulos
authors.forename: Philip
authors.initials: P
authors.lastname: Savage
authors.forename: James Richard
authors.initials: JR
authors.lastname: Smith
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
authors.forename: Mona A
authors.initials: MA
authors.lastname: El-Bahrawy
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism*; D002454:Cell Differentiation; D005260:Female; D005354:Fibrosarcoma / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007265:Inhibins / Q000378:metabolism; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010051:Ovarian Neoplasms / Q000378:metabolism / Q000473:pathology*; D010386:Pelvic Neoplasms / Q000378:metabolism / Q000473:pathology / Q000556:secondary*; D018312:Sex Cord-Gonadal Stromal Tumors / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D007265:Inhibins