Multiple Primary Intracranial Tumors in von Recklinghausen's Neurofibromatosis

Abstract

RECENTLY we have encountered an unusual case of central Recklinghausen's neurofibromatosis with neurofibromas of the cranial nerves and spinal nerve roots, a unilateral acoustic neurinoma, multiple cranial meningiomas, multiple ependymomas of the brain stem and cervical spinal cord, and syringomyelia of the cervical spinal cord. A study of the literature failed to reveal a review of the published cases of this type. The majority of the available reports present details of isolated cases with a discussion of possible etiological factors. A few reports give incomplete lists of similar cases. We wish to present our case with an analytical review of all the reported cases of neurofibromatosis associated with acoustic nerve neurinomas and multiple meningiomas. Report of a Case The patient was a 20-year-old white man who had intermittent fronto-occipital headaches for several years. Two years prior to admission, he developed paralysis of the left VII cranial nerve. One year