Diagnostic and clinical outcome of neurogenic tumours in the head and neck area.

A retrospective analysis of diagnostic procedures and clinical outcome of patients with neurogenic tumours of the head and neck region was performed. There were 25 patients - 16 with neurinoma, 5 with neurofibroma and 4 with neurogenic sarcoma - who presented with a mass in the head and neck area. In 13 patients, the neurinoma originated from the facial nerve, 2 of them were located in the middle ear and mastoid and showed facial palsy. Four patients presented with neurofibromatosis type 1. Twenty-three patients were operated on to remove the tumorous masses. Eighteen tumours were assigned intraoperatively to a cranial nerve main trunk, whereas 7 tumours originated from small branches or showed diffuse growth in neurofibromatosis. All benign neurogenic tumours were removed totally without relapse. One of 2 auricular nerve autografts in middle-ear facial nerve neurinoma resulted in a sufficient muscle tonus of the face. Three patients with malignancies died within 12-24 months showing local recurrencies (n = 1) and/or pulmonary metastasis (n = 2). As expected, neurogenic tumours are unusual neoplasms of the head and neck regions. On the other hand, neurogenic malignancies exhibit diagnostic problems as well as uncertain chances for long-term survival. Tumour staging, determined by extension and critical for treatment decisions, is well evaluated by CT scan and MRI. A multimodal therapy regimen with operation and radiation is recommended for neurogenic sarcoma also when developing in neurofibromatosis. However, in main trunk neurinomas, nerve autografting with interposition is the therapy of choice with a good chance of functional recuperation.