1. Schwannomatosis. An unusual variant of neurofibromatosis or a distinct clinical entity?
- Author
-
Purcell SM and Dixon SL
- Subjects
- Adolescent, Adult, Diagnosis, Differential, Female, Humans, Neoplasms, Multiple Primary complications, Nervous System Diseases etiology, Nervous System Neoplasms complications, Neurilemmoma complications, Neurofibromatosis 1 classification, Neoplasms, Multiple Primary classification, Nervous System Neoplasms classification, Neurilemmoma classification
- Abstract
Multiple schwannomas have frequently been seen in patients with neurofibromatosis. Recently, the association of multiple cutaneous schwannomas, central nervous system tumors, and various neurologic deficits has been described in Japanese patients as a condition called schwannomatosis. We describe the first non-Japanese cases of schwannomatosis and compare and contrast this unusual condition with the well-known variants of neurofibromatosis. We conclude that the features of schwannomatosis are distinct and define a condition that does not fit into the current classification scheme of neurofibromatosis. The occurrence of multiple cutaneous schwannomas in the absence of cardinal features of neurofibromatosis may indicate the presence of central nervous system tumors or various neurologic deficits.
- Published
- 1989
- Full Text
- View/download PDF