[A case of neurilemmomatosis]

Abstract

A 25-year-old man had multiple subcutaneous nodules on the neck, trunk and extremities. Those nodules first appeared in the right popliteal fossa about 15 years ago and thereafter gradually increased in the number with hearing loss, tinnitus, and vertigo. The tumors had smooth surfaces and were elastic hard without any adhesion to the overlying skin or to subcutaneous tissue. Histological examination revealed, in most parts of the lesion, Antoni type A neurilemmoma and the Antoni type B picture at the periphery. As extracutaneous lesion, there were bilateral cerebello-pontine angle neurilemmomas, and spinal and adrenal tumors. Similar subcutaneous tumors have been recognized in 6 of 17 family members of the previous four generations of his family. According to a statistical analysis of 119 cases, including this one, in the Japanese dermatological field, of 75 cases of solitary neurilemmoma and 44 cases of multiple ones, the latter showed male predominance and lower age of the onset. Many of them had tumors not only in the skin but also in extracutaneous sites.