Female Adnexal Tumour of Probable Wolffian Origin (FATWO): Review of the Literature

Abstract

FATWO (female adnexal tumour of probable Wolffian origin) denotes a rare tumour of the female adnexa which has been described in nearly 80 cases worldwide and which presumably originates in the remains of the Wolffian ducts. In 10 to 20 percent of patients, a metastasis or recurrence was seen subsequently and for this reason, a malignant potential is attributed to the FATWO. Because of the small number of cases, there is no clear therapeutic recommendation. The method of choice currently is surgical treatment with hysterectomy and adnexectomy. The benefit of radio- and chemotherapies is not clear and a small number of treatment attempts with imatinib have been made in cases of CD117 positivity or treatment attempts on an endocrine basis. This work provides an overview of the literature on epidemiology, imaging and histopathological diagnostic features as well as therapeutic options of this same tumour form.

Malignant recurrence of Female Adnexal Tumor of Probable Wolffian Origin (FATWO)

“…However, the National Comprehensive Cancer Network (NCCN) defines FATWO as borderline tumors with uncertain malignant potential ( Network and Clinical Practice Guidelines, 2020 ). About 10–20% of cases have been reported as malignant and/or recurrent ( Hubner et al, 2019 , Syriac et al, 2011 ). Recurrence is rare; the reported median time of tumor recurrence after initial resection is 48 months ( Syriac et al, 2011 , Hong et al, 2017 ).…”

“…Recurrence is rare; the reported median time of tumor recurrence after initial resection is 48 months ( Syriac et al, 2011 , Hong et al, 2017 ). Most frequent sites of metastasis are liver and lung ( Hubner et al, 2019 , Syriac et al, 2011 , Sheyn et al, 2000 , Shalaby and Shenoy, 2020 ).…”

“…Computed tomography (CT) can determine anatomic features and origin of tumor ( Shalaby and Shenoy, 2020 ). Immunohistochemical features and mutation analyses may aid in differentiating a FATWO tumor from other ovarian tumors, but are not diagnostic as can be seen in Table 1 ( Hubner et al, 2019 , Syriac et al, 2011 , Hong et al, 2017 ).…”

“… EMA, S100, and a-inhibin (−) cysplatin-cytoxan chemotherapy Hong et al, 2017 Malignant FATWO CA-125 = 70.3 CD10, CK7, and EMA (+) ER/PR (+) TAH, BSO mass resection, omentectomy, and left iliac and paraaortic lymphadenectomy. NA “close monitoring” HE4 = 147 CEA = 4.3 CEA, inhibin, CD99, Calretinin, CK20, WT-1, and PAX8 (−) Syriac et al, 2011 Recurrent FATWO NA C-kit (CD117), AE1/3, cytokeratin (CK)7, WT1, calretinin, and α-inhibin (+) NA TAH, BSO, omentectomy and bilateral pelvic and para -aortic lymph node dissection 3 year recurrence follow-up by CT every 6 months EMA) CK20, and synaptophysin (−) Imatinib mesylate therapy for 6 months Hubner et al, 2019 Benign FATWO Ca-125 = 23.1 CD10 and cytokeratins (the pancytokeratin antibodies AE1/3, Cam5.2, cytokeratin 7) PR (+) laparoscopic tumor removal and chromopertubation. NA NA CEA = 0.3 Inhibin (+) ER (−) irregular, diffuse reactivity for SF1 partial positivity for calretinin and CD56.…”

“…However, suggested therapy is tumor resection with total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO). In some cases of malignant Wolffian tumors, adjuvant chemotherapy and radiotherapy have demonstrated promising results with platinum and taxane based regimens ( Shalaby and Shenoy, 2020 , Network and Clinical Practice Guidelines, 2020 , Hubner et al, 2019 , Syriac et al, 2011 ).…”