Life-Threatening Intratumor Hemorrhage in Plexiform Neurofibroma: A Case Report and Literatures Review

Abstract

Plexiform neurofibromas is a common feature of neurofibromatosis 1 (NF1). This benign soft tissue tumor can cause different kinds of complications, depend on their size and location. Some of the complications demand prompt and aggressive surgical intervention, such as massive intratumor hemorrhage, which is potentially life-threatening if not treated adequately and can result in hypovolemic shock or compression of vital structures. We report a case of trunk plexiform neurofibromas with spontaneous massive intratumor hemorrhage, which could not be controlled under conservative management and resulted in chest wall compression, respiratory insufficiency as well as hypovolemic shock 18 hours after the episode. Urgent surgical intervention was performed and totally 6,000 ml blood clot and hematoma were evacuated from the tumor. He received angiography and selective embolization as an adjuvant therapy for further hemostasis. A huge skin defect developed over the fragile and myxomatous tumor-tissue secondary to ischemic change, which was successfully treated by vacuum-assisted wound closure system (VAC) followed by skin graft. The patient was doing well at 1-year follow-up.