Female adnexal tumour of probable Wolffian origin: a rare en
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A 39-year-old healthy woman, gravida 2 para 2, with an incidental sonographic diagnosis of a right adnexal solid mass. Transvaginal ultrasound revealed an enlarged fibroid uterus and a 51 mm nodular para ovarian mass suggesting either a pedunculated or a broad ligament fibroid. Blood tests showed normal serum cancer markers. At that time, the patient was considering getting pregnant again. Consequently, expectant management was decided.

On physical examination, the enlarged uterus and a solid nodular mass on the right adnexal area were palpable. On transvaginal ultrasound, the right adnexal mass appeared complex, measuring 115 mm×66 mm, with both solid and cystic components, and low vascularity in colour Doppler evaluation. The serum cancer antigen 125 (CA-125) level was 67.3 U/mL. A solid and cystic structure and increased serum CA-125 raised concern for ovarian cancer, but hypervascularity of the solid aspects suggested a fibroid or another benign tumor, with degenerative cystic changes. In this context, exploratory laparotomy with intraoperative pathology consultation was decided, confirming a 15 cm×8 cm×8 cm solid and cystic mass. The frozen section revealed a solid spindle cell pattern, favoring a fibrothecoma. Right salpingo-oophorectomy, total hysterectomy and left salpingectomy were performed. The tumour’s various morphologic patterns (predominantly solid, tubular and sieve-like) and Wolffian immunophenotype were consistent with a female adnexal tumour of probable Wolffian origin (FATWO)

After discussing management options with the patient at follow-up, and in the absence of established prognostic criteria, no further intervention was planned. The patient is clinically well (4 years post-operation). This challenging diagnosis requires thorough morphologic and immunohistochemical evaluation since there are no typical morphologic features nor a specific immunostain. These tumors are usually benign, but almost one-fifth of cases show more aggressive behavior, with distant metastases and recurrence even long after initial diagnosis. Due to the rarity of this condition—and consequent scarcity of data—its prognostic factors are not clearly established. From the limited information available, the prognosis does not seem to correlate unequivocally with either clinical presentation or histopathological features.

Source: https://casereports.bmj.com/content/13/10/e237127?rss=1