Brain tumors in hereditary multiple system hamartomatosis (tuberous sclerosis).

In this study brain tumors developing with tuberous sclerosis were biologically benign; total transcortical tumor removal permanently relieved the attending cerebral hypertension and achieved prolonged survival without notable functional impairment. In tuberous sclerosis, hamartomas characteristically develop from a random focal defect in organogenesis that affects many organ systems, sometimes seriously altering their function. Thus, genetic penetrance primarily determines the over-all prognosis, and "hereditary multiple system hamartomatosis" accurately describes the disease.