Malignant peripheral nerve sheath tumor of the uterine corpus presenting as a huge abdominal neoplasm

A 45-year-old P 3 L 3 , referred to us with abdominopelvic mass for further management. Vaginal examination was suggestive of uterine mass. Magnetic resonance imaging. (MRI) of abdomen.pelvis disclosed a uterine mass with equivocal invasion of the fat plane with the sigmoid colon. Coelomic antigen. (CA) 125 was 120.2 U/ml. (normal range, 0-35 U/ml). On exploratory laparotomy entire pelvic cavity was filled with a mass that was seen arising from the uterus and involving the sigmoid colon. Hence, a total abdominal hysterectomy with bilateral salpingo-oopphorectomy. (TAH BSO) was performed, along with resection anastomosis of the rectosigmoid and excision of omental and pelvic peritoneal nodules. Histopathology and immunohistochemical analysis, including S100-P positivity confirmed diagnosis of a malignant peripheral nerve sheath tumor. (MPNST), with tumor deposits in the right parametrium, omentum, sigmoid colon, and pelvic peritoneum. This case is presented in view of its rarity and associated diagnostic and therapeutic implications.