The case history of a woman with a "female adnexal tumor of probable Wolffian origin" is described in which the neoplasm recurred three times during a 16-year interval and responded with prolonged remission after repeated surgical excision and radiotherapy. Ultrastructural data are given to support the Wolffian nature of the tumor. Although the tumor was considered in the past to be a benign neoplasm, the current report, as well as six others in which the tumor recurred or metastasized, indicates that the female adnexal tumor of probable Wolffian origin should be classified within the International Classification of Disease-Oncology and the Systematized Nomenclature of Medicine nomenclatures as "of low malignant potential."
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