Extraovarian sex-cord-stromal tumors, especially fibrothecoma, are a rare entity and therefore lead to frequent diagnostic uncertainties. We report the case of a fibrothecoma originating from the right uterosacral ligament in an 83-year-old female presenting with mixed urinary incontinence due to a tumorous lesion absorbing the entire pelvis. The patient had previously undergone multiple pelvic surgeries including hysterectomy and bilateral adnexectomy. In a preoperative percutaneous biopsy the lesion was diagnosed initially as PEComa. After surgical extraction of the tumor and subsequent thorough histopathological, histochemical and immunohistochemical examination the initial diagnosis had to be changed to fibrothecoma. Diagnosis of extraovarian sex-cord-stromal tumors remains challenging and tight interdisciplinary exchange is necessary to improve diagnostics and consecutive therapy in patients with this rare tumor entity.
Geiger J, Montavon C, Glatz K and Heinzelmann-Schwarz V