Multiple schwannomas in the peripheral nerves

Multiple tumours of peripheral nerves are often seen in patients with neurofibromatosis of type 1 or 2. Multiple schwannomas may occur without other manifestations of neurofibromatosis. We have reviewed 12 patients with multiple schwannomas arising from peripheral lesions who did not fulfil the criteria for either type of neurofibromatosis. Four had spinal and one an intracranial lesion in addition to the peripheral tumours. Two patients had one and three café-au-lait spots, respectively, and another had a probable family history. The largest tumours were 45 to 250 mm in size. Three patients had been referred as having von Recklinghausen's disease. The large size of tumours, the difficulties of histological diagnosis on biopsy, and the confusion with neurofibromatosis can lead to overtreatment. Malignant change seldom, if ever, occurs in patients with multiple schwannomas.