Neurogenic tumors of the head and neck in children

Abstract

Soft tissue tumors make up 63% of all tumors in children. Tumors of the sympathetic chain make up 7.4% of tumors arising in children (nine new cases a year for every million children in USA) while neurofibrosarcomas make up 3.4% (2.4 new cases per year for every million children). There is a certain difference between the frequency of benign forms (rather elevated) and that of malignant forms (rather low). Diagnosis is possible by echo-scan, computed tomography, magnetic resonance imaging and fine-needle aspiration biopsy. As regards therapy, surgical resection represents the treatment of choice. In our experience, 35 neurogenic tumors in pediatric patients (8-16 years), arising in head and neck spaces, were observed and treated in the period 1976 and 1995. Twenty-six cases were schwannomas, six were neurofibromas and three were olfactory neuroblastomas. All the patients underwent surgery. Sacrifice of the affected nerve was necessary in 12 cases (all neurofibromas and eight neurinomas). In one case of olfactory aesthesioneuroblastoma a combined approach (extra-intracranial approach) was employed. Two patients are alive and disease-free with 5 and 7 years follow-up. As regards dysfunctional pathology following surgical resection, we report definitive facial nerve palsy in two cases, permanent laryngeal palsy in six cases, tongue dysfunction in one case and cheek hypoaesthesia in one case.