title: Pineal region giant cell astrocytoma associated with tuberous sclerosis: case report.
abstract: Tuberous sclerosis complex is a genetic disorder characterized by the development of hamartomas in multiple organs including the brain, skin, eye, kidney, and heart. Neurological features include seizures and mental retardation. Cortical tubers and subependymal nodules are the characteristic intracranial lesions of tuberous sclerosis. Subependymal giant cell astrocytomas, typically located adjacent to the foramen of Monro, can enlarge and cause symptomatic ventricular obstruction. The authors describe the case of a 3-year-old boy with a history of tuberous sclerosis and retinal lesions who presented with an enlarging enhancing pineal region mass. Via an infratentorial supracerebellar approach, the mass was removed using both the operative microscope and a rigid neuroendoscope. Pathological examination showed a giant cell astrocytoma. To the authors' knowledge, this is the first reported case of tuberous sclerosis associated with a giant cell astrocytoma of the pineal region. Diagnostic considerations are discussed.
authors.affiliation: Division of Pediatric Neurosurgery, Rainbow Babies and Childrens Hospital, Cleveland, Ohio 44106-5036, USA.
authors.forename: Shervin R
authors.initials: SR
authors.lastname: Dashti
authors.forename: Shenandoah
authors.initials: S
authors.lastname: Robinson
authors.forename: Mark
authors.initials: M
authors.lastname: Rodgers
authors.forename: Alan R
authors.initials: AR
authors.lastname: Cohen
mesh_terms: D001254:Astrocytoma / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D002675:Child, Preschool; D005124:Eye Abnormalities / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D005500:Follow-Up Studies; D006801:Humans; D007089:Image Enhancement; D008279:Magnetic Resonance Imaging; D008297:Male; D008866:Microsurgery; D044582:Neuroendoscopes; D009460:Neurologic Examination; D010870:Pineal Gland / Q000473:pathology / Q000601:surgery; D010871:Pinealoma / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D011183:Postoperative Complications / Q000175:diagnosis; D014402:Tuberous Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000473:pathology