Extragenital malignant mixed Mullerian tumor

Abstract

Reports of extragenital malignant mixed Mullerian tumor (MMMT) type though extremely rare have increased. This represents the 15th reported case of MMMT outside the female reproductive tract. A 68-year-old black woman presenting with abdominal pain, increasing abdominal girth, and a provisional diagnosis of ovarian carcinoma, underwent exploratory laparotomy, total abdominal hysterectomy, omentectomy and cytoreductive surgery. Surgery and histology revealed MMMT of heterologous type in the pelvic peritoneum, with no tumor in the genitalia and no foci of endometriosis. Despite surgical debulking leaving residual tumor of less than 1 cm followed by aggressive systemic chemotherapy, the patient died of disease within 2 months. Increasing exploratory surgery may account for the increase in reports of extragenital MMMT. Aggressive tumor debulking and multiagent systemic chemotherapy is of limited efficacy since extragenital MMMT, like uterine MMMT with pelvic spread, has short survival.