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October 7, 1961

Tuberous Sclerosis Complex and Astrocytoma: Report of a Case with Successful Removal of the Neoplasm

Author Affiliations


Under assignment by the U.S. Public Health Service, Division of Radiologic Health, to the Atomic Bomb Casualty Commission, Hiroshima-Nagasaki, Japan (a Research Agency of the U.S. National Academy of Sciences and National Research Council under a grant from the U.S. Atomic Energy Commission administered in cooperation with the Japanese National Institute of Health of the Ministry of Health and Welfare) (Dr. Rudnick); and Professor of Surgery, Department of Neurosurgery (Dr. Hoshino), and Department of Neurosurgery (Dr. Kitaoka), Hiroshima University Medical School. Dr. Rudnick is now at the Peter Bent Brigham Hospital, Department of Internal Medicine, Boston.

JAMA. 1961;178(1):73-75. doi:10.1001/jama.1961.73040400026017d

THE DEVELOPMENT of malignant gliomata in patients with tuberous sclerosis is an occasional feature of this hereditary disease.1 Recently 2 patients in the same family who had tuberous sclerosis and intraventricular astrocytomas were reported.2 Ependymoma3 and spongioneuroblastomas4 also have been found in patients with this disease. However, those patients with tuberous sclerosis who have symptoms of intracranial neoplasm usually develop these symptoms because of critically situated cortical and subependymal nodules characteristic of this disorder.5

Pulmonary,6 renal,7 and osseous manifestations8,9 of tuberous sclerosis have been described in addition to the classical triad of epilepsy, mental retardation, and adenoma sebaceum. Knowledge of the wide-spread involvement and of the various clinical syndromes due to tuberous sclerosis has made it possible to diagnose this disease correctly and more frequently.

Blindness secondary to retinal gliomatous tumors known as phacomas has been observed.10 Also blindness might be