Rapidly growing diffuse neurofibroma of the scalp with calvarial defect in a young woman: A rare entity

Abstract

Background: A diffuse neurofibroma, a variant of neurofibroma, most commonly occurs in young adults and involves the head and neck. In the absence of neurofibromatosis, associated calvarial defect with these swellings is rarely seen.Case Description: An 18-year-old woman presented with a history of rapidly progressive painless large swelling over the bilateral parieto-occipital region of scalp. It was soft and boggy with brownish discoloration of overlying skin. Imaging study showed brilliantly enhancing diffuse lesion involving the bilateral parieto-occipital region of scalp and extending into the extradural region. She underwent excision of lesion. Postoperatively, she developed flap necrosis and it was allowed to heal with the secondary intention. The biopsy findings were consistent with neurofibroma. The patient is on regular follow-up, without any evidence of recurrence at 1 year.Conclusion: Rapidly growing solitary diffuse neurofibroma is rare in children and adolescents. Preoperative diagnosis may be difficult and surgical treatment needs to be individualized. These patients need regular follow-up for early detection of recurrence.