MALIGNANT MIXED MULLERIAN TUMOR OF THE EXTRAOVARIAN SECONDARY MULLERIAN SYSTEM - REPORT OF 2 CASES AND REVIEW OF THE ENGLISH LITERATURE

Objective.-To report two cases of malignant mixed mullerian tumor of the extraovarian secondary mullerian system and to identify cases reported in the English literature. Design.-Two cases are described and discussed along with cases reported previously in the English literature. Setting.-The University of Cincinnati Medical Center. Patients.-(1) A 62-year-old woman with a bilateral ovarian poorly differentiated endometrioid adenocarcinoma with multiple peritoneal metastasis and a malignant mixed mullerian tumor with heterologous elements arising from the pelvic peritoneum. (2) An 83-year-old woman with a malignant mixed mullerian tumor with heterologous elements arising from the cecal peritoneum. Results.-Seventeen previously reported cases were identified in the English literature. Conclusions.-The malignant mixed mullerian tumor of the extraovarian secondary mullerian system is a rare disease with only 17 cases reported to date to our knowledge. The prognosis is poor. Of 12 patients with follow-up information available, 10 died within 1 year and 2 within 2 years after diagnosis. The histogenesis of the tumor remains unclear; however, data exist that support transformation of epithelial neoplastic cells into sarcomatous cells (metaplastic theory) and origin from a single totipotential cell. It is possible that both situations may occur.