Brain Tumors with Tuberous Sclerosis

Abstract

I N CONTRAST to the high incidence of brain tumors in pa t ien ts with yon Reckl inghausen 's disease, intraeranial tumors are seldom seen in pat ients with tuberous sclerosis. The present confusion regarding the pa tho logy and preferred therapy for these lesions can pe rhaps be a t t r ibuted to the limited n u m b e r of eases tha t a single neurosurgeon or pathologis t is likely to encounter, and the fact t h a t the related literature is composed main ly of single case reports. The te rm bra in t u m o r , when applied to a lesion in tuberous sclerosis, mus t be carefully defined, for s t r ict ly speaking, each of the multiple in t ravent r icu la r nodules tha t characterist ically occur in this disease could be considered a tunmr . In this report, therefore, the word t u m o r will be reserved for those lesions associated with increased intracranial pressure. In a recent review of pat ients a t Duke Universi ty Medical Cente r and the menta l hospitals serving Eas t e rn N o r t h Carolina, 48 cases of tuberous sclerosis were found. Of these 48 cases, seven had intracranial tumors. Five of these were verified a t operation, one a t au topsy , and one by ventriculography. The clinical and pathological findings, and our experience in t reat ing these seven patients , are summar ized in this report . The reader is referred to discussions of the cutaneous, 2 retinal, 14 cardiac, 1 pulmonary, 5 renal, s and bone 6 lesions of tuberous sclerosis.