Female Adnexal Tumor of Probable Wolffian Origin: A Review

Abstract

Context.— Female adnexal tumor of probable Wolffian origin (FATWO) is an extremely rare gynecologic neoplasm of low malignant potential. Fewer than 90 cases of this entity have been described in the English-language literature. It is presumed to be derived from mesonephric (Wolffian) duct remnants in the upper female genital tract. We provide a literature review to increase awareness of this extremely uncommon entity. Objectives.— To review the clinical and pathologic findings of FATWO and to discuss common entities in the differential diagnosis. Data Sources.— The study involved PubMed (National Center for Biotechnology Information, Bethesda, Maryland) searches, including multiple review articles, case reports, retrospective studies, selected book chapters, and University of Mississippi Medical Center cases. Conclusions.— FATWO can affect patients from a wide age range and present with a nonspecific clinical presentation. It typically presents as solid tumors with occasional nodular, lobulated, or cystic appearances. FATWO can show a variety of histologic patterns which may result in diagnostic difficulties for pathologists. There is no single specific immunohistochemical stain for FATWO, and the pathogenesis and molecular alterations are not yet well understood. Although it is generally considered a benign entity, recurrent and metastatic cases have been reported. There are no current recommendations regarding the optimal clinical management of FATWO.

CHEK2 Mutation in Patient with Multiple Endocrine Glands Tumors. Case Report

“…FATWO is a very rare gynecologic neoplasm of low malignant potential, with fewer than 90 cases being reported worldwide [22]. It is presumed to be derived from the mesonephric (Wolffian) duct remnants in the upper female genital tract [23]. Moreover, some recurrent and metastatic cases have been reported in approximately 11% of cases [24].…”

Female adnexal tumour of probable Wolffian origin: a rare entity with challenging histopathological diagnosis and unpredictable behaviour

“…2 FATWOs are most frequently found in the broad ligament, but can also appear in the mesosalpinx, Fallopian tube, ovary, paravaginal region or peritoneum. 3 The median age at diagnosis is 50 years (range 15-87 years). 2 3 FATWOs can resemble ovarian endometrioid adenocarcinomas (tubular and sieve-like patterns), Sertoli-Leydig cell tumours (tubular or retiform patterns) or granulosa stromal cell tumours, namely of the fibroma-thecoma group (solid pattern), since they show variable intertumoural and intratumoural morphology (mostly solid, tubular and sieve-like).…”

“…6 From the limited information available, prognosis does not seem to correlate unequivocally with either clinical presentation or histopathological features. 3 Consequently, treatment is individualised. Generally, the most adequate treatment is considered to be total surgical resection, with…”

“…tumour debulking, total hysterectomy and bilateral salpingooophorectomy. 3 The role of complementary treatments is controversial. 3 Due to its malignant potential, regular and long-term follow-up is recommended.…”

“…3 The role of complementary treatments is controversial. 3 Due to its malignant potential, regular and long-term follow-up is recommended. 6…”