Massive scalp hematoma due to diffuse neurofibroma in NF-1

摘要

Neurofibroma is a commonly occurring soft tissue tumor. The focal variety is the commonest, plexiform is pathognomic of NF-1 and diffuse is the one most commonly seen in young adults in the head and neck region. The diffuse variety is only seen in a minority of NF-1 cases. Scalp neurofibroma in itself is a relatively rare presentation in patients with NF-1 [1]. Massive bleed in scalp neurofibroma is very rare and till date only 5 cases have been reported [2]. Diffuse neurofibroma is notknown to bleed despite of increased vascularity. Resection is carried out in case of severe disfigurement or functional impairment. Total excision is difficult because of extensive and infiltrative nature of the lesion. The incidence of a rebleed in a neurofibroma is not known. Cases with rebleed, some occurring as late as 30 years have been reported [3]. The treatment includes preoperative embolization to reduce the blood loss followed by excision and reconstruction. Here a case of NF-1 with diffuse scalp neurofibroma with massive bleed, requiring evacuation is presented.