Aims: To establish the prognosis associated with female adnexal tumour of probable wolffian origin (FATWO) and determine the frequency with which it behaves as a truly benign lesion.
Methods: Medline and Embase electronic databases were interrogated to identify 31 papers describing 63 patients with FATWO with follow up.
Results: Fifty (79%, CI 67.4-87.3%) were alive and well but seven patients had recurrent or residual disease (11.1%, CI 5.6-21.5%) and three had died of disease (4.8%, CI 1.6-13.1%). Stage (p = 0.0002) and differentiation (p = 0.0118) showed a significant association with outcome although atypia approached significance at the 5% level (p = 0.0658). One patient with a ruptured tumour had recurrent disease and one other had died of disease (p = 0.2278). There was no association between outcome and age (p = 0.6651), size (p = 0.1912), length of survival (p = 0.2351) tumour site, mitotic rate or necrosis (p = 0.5937, 0.4697 and 0.2016, respectively).
Conclusion: On the basis of these findings, FATWO cannot be regarded as a benign lesion. These lesions may be confused with well differentiated gynaecological cancers and careful clinicopathological correlation with the extensive use of immunohistochemistry is encouraged to ensure that lesions such as extragonadal endometrioid adenocarcinoma is not confused with FATWO.