Multiple tumours of the peripheral nerves are seen only in neurofibromatosis. They are hereditary. They present and develop in a variety of different ways. Three main groups are distinguished: von Recklinghausen neurofibromatosis or type 1; bilateral acoustic neurofibromatosis or type 2 and schwannomatosis recently defined as type 3. The aim of this study was to clarify the clinical outcome of neurofibromatosis. The diagnosis is made purely on clinical grounds. Cranial MRI and slit lamp examination are useful for classification. Surgical management for peripheral nerve tumours is similar. Any new and rapid change noted at clinical examination (increase in volume, pain or neurological deficit) requires surgery because of potential malignant transformation of the neurofibroma into neurofibrosarcoma (type 1 only). The definitive treatment depends on the resectable character of the tumour which is usually only known after epineurotomy under operating microscope. In the event of resectable tumour (schwannoma) enucleation must be performed, preserving nerve continuity. In the event of unresectable tumour (neurofibroma), tumour resection is impossible without sacrificing nerve tissue. An epineurotomy must be performed. It prevents further deterioration. Interfascicular biopsy confirms the histological type. Our results are similar to those in other recorded studies. The unpredictable clinical course of neurofibromatosis makes prolonged follow-up mandatory.
