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Female Adnexal Tumor of Probable Wolffian Origin (FATWO) With Recurrence 3 Years Postsurgery

Syriac, Susanna M.D.; Durie, Nicole M.D.; Kesterson, Joshua M.D.; Lele, Shashrikant M.D.; Mhawech-Fauceglia, Paulette M.D.

International Journal of Gynecological Pathology: May 2011 - Volume 30 - Issue 3 - p 231–235
doi: 10.1097/PGP.0b013e3182005340
PATHOLOGY OF THE UPPER GENITAL TRACT: CASE REPORT
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This is the case report of a 38-year-old woman who presented with a mass of the right broad ligament that was diagnosed as a female adnexal tumor of probable Wollfian origin (FATWO). The patient was treated with a simple mass excision. Three years after the excision, the patient presented with uterine bleeding. A total abdominal hysterectomy was advised. Intraoperative histologic consultation showed a poorly differentiated tumor on the surface of the left ovary. After extensive immunohistochemistry analysis and after reviewing the histology slides from the primary tumor, the final diagnosis was concluded to be recurrent FATWO on the surface of the ovary. C-kit immunohistochemistry was found to be strongly positive. Polymerase chain reaction amplification of C-kit genes on exons 9, 11, 13, and 17 and of PDGFR gene on exons 12 and 18 showed no mutational changes. Owing to the limited options in treating recurrent disease and the lack of prognostic factors for recurrence or metastasis, the patient was started on 400 mg of imatinib mesylate therapy for 6 months. In addition, the patient is undergoing continuous follow-up by computed tomographic imaging every 6 months. As chemotherapy and radiation therapy for recurrent or metastatic FATWO are most often unsuccessful, a molecular targeted therapy, such as tyrosine kinase inhibitor, could be considered. However, collective data are needed from multiple centers to determine its effectiveness in these patients.

Department of Surgical Pathology (S.S., N.D., P.M-F.)

Department of Gynecology-Oncology Surgery (J.K., S.L.), Roswell Park Cancer Institute, Buffalo, NY

Address correspondence and reprint requests to Paulette Mhawech-Fauceglia, MD, Department of Pathology, Roswell Park Cancer Institute, Elm and Carlton Street, Buffalo, NY 14263. e-mail: pmhawech1@yahoo.com; e-mail: Paulette.mhawech-fauceglia@roswellpark.org

©2011International Society of Gynecological Pathologists