[Neurilemmomatosis--a sporadic and familial cases].

Abstract

Three cases of neurilemmomatosis are reported. A 22-year-old man without any relatives with similar symptoms visited our clinic, complaining of multiple skin tumors since the age of 15 and bilateral acoustic nerve symptoms since 19. Physical examination revealed no pigmented or depigmented spots. Histopathological examination of the eight tumors excised from the skin, acoustic nerve and spinal cord showed that these were all neurilemmomas. A 36-year-old man with a 15 year history of multiple skin tumors and one year history of acoustic nerve symptoms was seen at our clinic, revealing no pigmentary disorders. The tumors excised from the skin and bilateral acoustic nerves were all neurilemmomas histopathologically. A 5-year-old boy, who was the only child of the second case and had had several skin tumors since his birth, visited us after postoperative death of his father. He revealed no pigmentary abnormalities. The histology of the skin tumor was neurilemmoma. The absence of neurofibromas and pigmented spots in these patients with neurilemmomatosis suggests that this disorder might be close to, but distinct from neurofibromatosis. Although familial cases of neurilemmomatosis like our case 2 and 3 reported so far are very few, they support a possibility that neurilemmomatosis might be a genetically determined neurocutaneous syndrome, a kind of phacomatosis.