Malignant mixed müllerian tumor of the extraovarian secondary müllerian system. Report of two cases and review of the English literature

Abstract

Objective: To report two cases of malignant mixed müllerian tumor of the extraovarian secondary müllerian 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 müllerian tumor with heterologous elements arising from the pelvic peritoneum. (2) An 83-year-old woman with a malignant mixed müllerian tumor with heterologous elements arising from the cecal peritoneum.

Results: Seventeen previously reported cases were identified in the English literature.

Conclusions: The malignant mixed müllerian tumor of the extraovarian secondary müllerian 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.