Abstract
Neurofibromas are benign peripheral nerve tumors composed of proliferating Schwann cells and fibroblasts. It has been estimated that approximately 25% of all neurofibromatosis (NF) are found in the head and neck. Patients with NF type 1 of the head and neck suffer from one of the most dramatic cosmetic disabilities, which may lead to psychological troubles and social segregation. There is no known complete cure for NF. Solitary neurofibroma is a rare tumor of the head and neck region. It is more common in viscera, where it is associated with features of NF1. It occurs most often between the third and fourth decade. These lesions are extremely rare in children. We report a case of giant, solitary neurofibroma presenting as a progressive mass in neck region in an child. This case is unique in its age of presentation 8 years, over neck on left side, size 12*11*10 cms without any symptoms or neurological deficit. We are presenting our experience in treating this case, with a trial to establish a protocol for dealing with such case as regards; collection and sorting them, assessment of the lesion, dealing with the complications and the plan of treatment. Difficulties encountered during surgery were discussed in particular the management of intraoperative bleeding and post operative brachial plexus neuropraxia.
Highlights
The aetiology behind solitary neurofibroma is still unknown
Plexiform neurofibromas (PNF) are benign tumors which originate from nerve sheath cells, subcutaneous, or visceral peripheral nerves that can involve multiple fascicles
PNF usually occur in as much as 30% of patients with neurofibromatosis type I (NF-I) – an autosomal dominant disorder caused by defect of one allele of the tumor suppressor gene, NF1 on 17q and encodes neurofibromin, a tumor-suppressor protein with GTPase-activating activity that inhibits RAS function
Summary
The aetiology behind solitary neurofibroma is still unknown. Marocchio et al.[1] considered solitary neurofibroma to be hyperplastic hamartomatous malformations rather than neoplastic. Neurofibromas are benign peripheral nerve tumors composed of proliferating Schwann cells and fibroblasts. They present as multiple, palpable, rubbery, cutaneous tumors. Plexiform neurofibromas (PNF) are benign tumors which originate from nerve sheath cells, subcutaneous, or visceral peripheral nerves that can involve multiple fascicles. Because of the involvement of multiple fascicles of nerves and tissues and the spread of PNF, there is high risk of neurological and functional destruction when surgical resection is carried out. PNF usually occur in as much as 30% of patients with neurofibromatosis type I (NF-I) – an autosomal dominant disorder caused by defect of one allele of the tumor suppressor gene, NF1 on 17q and encodes neurofibromin, a tumor-suppressor protein with GTPase-activating activity that inhibits RAS function
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