Diagnostic and Clinical Outcome of Neurogenic Tumours in the Head and Neck Area

Abstract

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.

Neurofibroma of the Pinna

“…Neurofibromas may occur in isolation or as a part of Von Recklinghausen's syndrome in combination with café au lait skin patches, lisch's nodules, axillary freckling and fibroma molluscum [2]. The disease may be inherited as an autosomal dominant trait with variable penetrance in 50% of patients or it may occur as a result of spontaneous mutation [3].…”

“…Most tumours caused by NF need no treatment, but tumours that are painful, disfiguring, growing rapidly or impairing function need treatment. In main trunk neurofibroma, nerve autografting with interposition is therapy of choice with good chance of functional recuperation [3]. Large lesion may require reconstruction and elevation of pinna with dermal strips or marlex mesh [4].…”

Neurofibroma of the auriculotemporal nerve

“…The nerve of origin cannot be identified without surgical exploration [1,[4][5][6][11][12][13]. Ultrasonography permits direct visualization of the vagus nerve, distinction between schwannomas of the cervical sympathetic chain and schwannomas of the vagus nerve and a relationship to surrounding blood vessels [11]. In our patient the history of pain and the topography of the lesions (using CT and MRI) suggested that the auriculotemporal nerve was the nerve of origin but definitive assessment only was provided by surgical exploration.…”

“…Radical resection is only indicated if there is certainty that no motor nerves (such as the facial nerve) are affected, as reconstruction of significant motor nerves may produce incomplete functional recovery [11,13].…”

“…Neurofibromatosis due to von Recklinghausen's disease (central type II and peripheral type I) may present clinically as multiple neurofibromas, but solitary neurofibromas can also be the first manifestation of general peripheral neurofibromatosis [2]. Malignancy rarely occurs in solitary neurofibromas but has been observed in 3-30% of cases with von Recklinghausen's neurofibromatosis [4,7,9,11,12,14]. Such malignancy is thought to be stimulated by hormone imbalances associated with puberty [7].…”