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: Jayson Wang, Panagiotis Papanastasopoulos, Philip Savage, James Richard Smith, Cyril Fisher, Mona A El-Bahrawy
• Journal: International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists (Int J Gynecol Pathol) Vol. 34 Issue 4 Pg. 363-8 (Jul 2015) ISSN: 1538-7151 [Electronic] United States
• PMID: 25760903 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Biomarkers, Tumor
• Inhibins

Topics

• Biomarkers, Tumor (metabolism)
• Cell Differentiation
• Female
• Fibrosarcoma (metabolism, pathology)
• Humans
• Immunohistochemistry
• Inhibins (metabolism)
• Middle Aged
• Neoplasm Recurrence, Local
• Ovarian Neoplasms (metabolism, pathology)
• Pelvic Neoplasms (metabolism, pathology, secondary)
• Sex Cord-Gonadal Stromal Tumors (metabolism, pathology)