Intraventricular tumors in tuberous sclerosis

Eleven of our own cases of tuberous sclerosis with intraventricular tumor and 34 cases from the literature are reviewed. Initial symptoms and identification of tuberous sclerosis and tumor are reviewed. Results of surgical treatment, including direct radical excision and shunting are given. On gross examination, the tumor was most often loosely connected to ependyma near the foramen of Monro, causing hydrocephalus. Giant cell subependymal astrocytoma was frequently found on microscopic examination. The glial origin of the tumor is discussed and direct surgical approach of the tumor is proposed as the best treatment of these lesions.