Highlights Mesonephric-like adenocarcinomas (MLA) of the ovary are rare, require differentiation from other histiotypes. True histiogenesis of MLA of ovary remains debated, possibly arise from mullerian lesions of the ovary. MLA of ovary have differing genotypes from true mesonephric carcinomas of the cervix. Case described reflects further evidence for mullerian origin of MLA of ovary, with endometriosis in affected ovary. MLA of ovary can act aggressively, case described recurred and responded only partially to chemotherapy.

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