Abstract
CONTEXT: Female adnexal tumors of probable Wolffian origin (FATWO) represent very rare borderline ovarian tumors with low malignant potential. Only 15 cases of malignant FATWO are described in the current literature, among which, only 5 are reported as being recurrent. OBJECTIVE: Due to the rare presentation of the recurrence of the malignant FATWO and the few cases reported in the scientific database, there are no clear therapy recommendations. This paper should help practitioners to choose the best therapy approach. DESIGN: This paper presents the 6(th) case of malignant recurrent FATWO and will compare all the cases available in the literature. SUBJECTS AND METHODS: We present a review of the literature comparing the therapeutic approaches and outcomes of all the five cases of recurrent malignant FATWOs. Also, we introduce the case of a stage III Wolffian origin adnexal tumor with multiple recurrences appeared after 6 years of disease free interval. RESULTS: Our case presents the longest survival reported in the literature and underwent most surgical procedures of the recurrences and more than 4 lines of chemotherapy regimens. CONCLUSIONS: This paper shows possible therapeutic approaches to be used as example by the practitioners according to the drug availability in their centers.