DOI: 10.36347/sjmcr.2021.v09i10.011

Pages: 966-970

Primary malignant schwannomas are uncommon neoplasms of nerve sheath origin, particularly in the head and neck, where few cases have been documented in the literature. In the work-up of a neck mass, these tumors may offer a diagnostic dilemma and carry a poor prognosis despite wide excision, chemotherapy, and radiotherapy. We report the case of a 4 years old boy who had clinical evidence of neurofibromatosis type 1 and who presented with an asymptomatic solitary left cervical swelling. He was evaluated with an MRI scan of the neck. A biopsy was performed, and the anatomopathological and histologic examination revealed a plexiform and myxoid schwannoma. The tumor was deemed unresectable by the department of oral and maxillofacial surgery. Pathological and radiological evaluation, differential diagnosis of this neoplasm and its management are discussed.