A case of malignant mixed Müllerian tumor of the uterus with prominent neuroectodermal differentiation is reported. The most notable feature of the tumor was its large component of primitive neuroepithelium lining tubules. The islands of neuroectodermal tissue were admixed with conventional endometrioid adenocarcinoma and sarcomatous elements resembling malignant fibrous histiocytoma only in a small focus.
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