ABSTRACT

Neurofibromatosis (NF) type 1 is a hereditary, autosomal dominant neurocutaneous syndrome often accompanied by diffuse neurofibromas in the body, increased skin pigmentation (cafe-au-Iait spots) and central nervous system tumors. Neurofibromas are tumors of nerve sheath origin, composed of Schwann cells, perineural cells and fibroblasts. Neurofibromas of the scalp are rare findings of NF. Being a form of neurofibroma, diffuse neurofibroma shows marked dermal and subcutaneous thickening. In most adults, it is localized in the head, neck and body. A neurofibroma located in the scalp without NF or phakomatoses rarely generates a calvarial defect. In this report, we present a case of NF type 1 diagnosed after a bleeding scalp neurofibroma due to a head trauma. Gross total surgery is important in NF type 1 cases with a neurofibroma on the scalp. Especially in large lesions, a hemorrhage may occur within the lesion after head trauma. In these patients, computed tomography and magnetic resonance imaging of the brain should absolutely be performed before surgery. If the bone is destructed, cranioplasty should be considered in surgical planning. In the postoperative period, close follow-up for malignant transformation and recurrence is needed.