A Bilateral Wolffian Adnexal Tumor with Malignant Behavior: A Rare Case Report with Literature Review

Wolffian adnexal tumors (FATWOs) originate from the mesonephric duct remnants. FATWOs are extremely rare and 100 incidental FATWOs have been reported in the English literature as of now. Most FATWOs have low potential for malignancy but aggressive behavior including recurrence and metastasis have been described in few cases; There is no standard protocol for optimal treatment of FATWOs. The case described here is a 35-year-old female who presented with a right-side ovarian mass via abdominal ultrasound. She had a history of left salpingo-oophorectomy due to an abdominal mass, which both histopathologic and immunohistochemical study's findings were consistent with Wolffian tumor. Later, she underwent total abdominal hysterectomy with tumor debulking because of the probable malignant behavior of the tumor. FATWO has a heterogeneous histologic pattern which may make its diagnosis challenging. No specific immunohistochemical markers have yet been recognized for FATWO and pathogenesis or molecular alterations are not definitive. Therefore, there is no comprehensive recommendation for optimal clinical management of FATWO or its recurrence.