Ovarian Müllerian Adenosarcoma

Abstract

A 46-year-old woman with Von Recklinghausen disease was found to have a left ovarian mass. She was subjected to total abdominal hysterectomy with bilateral salpingo-oophorectomy. On section, the tumor was cystic with a polypoid solid portion protruding into its cavity. Microscopically, it was a müllerian adenosarcoma with a low-grade stromal component arising in ovarian endometriosis, with no heterologous elements. The patient underwent postoperative radiotherapy and chemotherapy. One year later, a brain metastasis was found localized in the left thalamus and was treated by gamma-knife radiosurgery. Fifteen months later, the brain tumor recurred, and the patient received a second treatment with gamma-knife radiosurgery. No recurrence in the pelvis was found. Six years postoperatively, the patient is free of disease.