J Korean Neurosurg Soc.  1989 Dec;18(7-12):1088-1092.

Clinical Experience of the Neurofibromatosis: Report of Three Cases

• Tsang YS
• Chung HS
• Choi CS
• Ju MB

Abstract

The usual form of neurofibromatosis is characterized by cafe-au-Iait skin pigmentation and pedunculated fibrous skin tumors but in the severe form, malformations and tumors of the central nervous system may be associated with von Recklinghausen's disease. Schwannoma of the spinal sensory roots, of the vestibular part of 8th cranial nerve, and of the sensory root of the 5th cranial nerve are particularly common. Outside the central nervous system, other neuroectodermal tumors may develop including renal, pancreas, and suprarenal tumors. Recently, neurofibromatosis is subdivided into type 1 and 2, according to the site of involvement. Type 1 is classic von Recklinghausen's disease, and type 2 represents bilateral acoustic neurinomas. The authors experienced 2 cases neurofibromatosis type 1 and 1 case of neurofibromatosis type 2 in which neurofibromas involve both cerebello-pontine angles, multiple levels of spinal cord and cauda equina.

Keyword

Neurofibromatosis; Type 1 and 2

MeSH Terms

Cauda Equina
Central Nervous System
Cranial Nerves
Neurilemmoma
Neuroectodermal Tumors
Neurofibroma
Neurofibromatoses*
Neurofibromatosis 1
Neurofibromatosis 2
Pancreas
Skin
Skin Pigmentation
Spinal Cord