Abstract
MPNSTs (malignant peripheral nerve sheath tumours) arise rarely from the female genital tract, even more rarely from the uterus. A literature review showed 18 cases affecting the uterine cervix, but only four affecting the uterus per se, making our present case the fifth to be involving the uterus, specifically round ligament. It was the first time a uterine lesion (not uterine cervix) was defined in a neurofibromatosis patient. This was a young patient who initially underwent a uterus-sparing surgery for a round ligament tumour which was finally diagnosed to be MPNST. She received adjuvant chemotherapy but recurred with pelvic vascular involvement. A challenging surgery was performed, and the tumour was resected without morbidity. She was followed up with radiotherapy; however, she quickly developed extensive peritoneal disease and succumbed to the disease. High-grade nature of MPNST along with the background of NF could have made the tumour more aggressive, highlighting the importance of suspecting MPNST in spindle cell tumours of pelvis and performing total resection in the first setting.