title: Brain tumors in hereditary multiple system hamartomatosis (tuberous sclerosis).
abstract: 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.
authors.affiliation: Shawnee Mission Medical Circle, Kansas 66202, USA.
authors.forename: J R
authors.initials: JR
authors.lastname: Cooper
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D001254:Astrocytoma / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D001932:Brain Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D002552:Cerebral Ventricles / Q000601:surgery; D002648:Child; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D005870:Giant Cell Tumors / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D006223:Hamartoma Syndrome, Multiple / Q000150:complications* / Q000235:genetics*; D006261:Headache / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods; D010672:Phenytoin / Q000627:therapeutic use
chemical_list: D000927:Anticonvulsants; D010672:Phenytoin