Ovarian malignant mixed mullerian tumor with primitive neuroectodermal differentiation: Case report with review of the literature

Ovarian malignant mixed mullarian tumor (OMMMT) is a rare and aggressive tumor of the female genital tract, occurring mainly in elderly women. Stage of disease is the most important predictor for survival with no prognostic effect, yet, of heterologous elements. Rare case reports described the peculiar presence of primitive neuroectodermal tissue among other heterologous elements in these tumors. Attractive designations, such as teratoid carcinosarcoma, were set by some authors to describe this subset of lesions, where it was considered a primary neuroectodermal tumor capable of multilineage differentiation. We here report a case of OMMMT in an elderly woman with focal primitive neuroectodermal differentiation as the sole heterologous element, and review the controversy on this topic in the literature.