title: Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012.
abstract: BACKGROUND
  Tuberous sclerosis complex is an autosomal dominant disorder predisposing to the development of benign lesions in different body organs, mainly in the brain, kidney, liver, skin, heart, and lung. Subependymal giant cell astrocytomas are characteristic brain tumors that occur in 10% to 20% of tuberous sclerosis complex patients and are almost exclusively related to tuberous sclerosis complex. Subependymal giant cell astrocytomas usually grow slowly, but their progression ultimately leads to the occlusion of the foramen of Monro, with subsequent increased intracranial pressure and hydrocephalus, thus necessitating intervention. During recent years, secondary to improved understanding in the biological and genetic basis of tuberous sclerosis complex, mammalian target of rapamycin inhibitors have been shown to be effective in the treatment of subependymal giant cell astrocytomas, becoming an alternative therapeutic option to surgery.
  
  
  METHODS
  In June 2012, an International Tuberous Sclerosis Complex Consensus Conference was convened, during which an expert panel revised the diagnostic criteria and considered treatment options for subependymal giant cell astrocytomas. This article summarizes the subpanel's recommendations regarding subependymal giant cell astrocytomas.
  
  
  CONCLUSIONS
  Mammalian target of rapamycin inhibitors have been shown to be an effective treatment of various aspects of tuberous sclerosis complex, including subependymal giant cell astrocytomas. Both mammalian target of rapamycin inhibitors and surgery have a role in the treatment of subependymal giant cell astrocytomas. Various subependymal giant cell astrocytoma-related conditions favor a certain treatment.
authors.affiliation: Department of Pediatric Neurosurgery, Dana Children's Hospital, Tel-Aviv Medical Center, Tel-Aviv, Israel. Electronic address: jonaroth@gmail.com.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Roth
authors.forename: E Steve
authors.initials: ES
authors.lastname: Roach
authors.forename: Ute
authors.initials: U
authors.lastname: Bartels
authors.forename: Sergiusz
authors.initials: S
authors.lastname: Jóźwiak
authors.forename: Mary Kay
authors.initials: MK
authors.lastname: Koenig
authors.forename: Howard L
authors.initials: HL
authors.lastname: Weiner
authors.forename: David N
authors.initials: DN
authors.lastname: Franz
authors.forename: Henry Z
authors.initials: HZ
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D001254:Astrocytoma / Q000175:diagnosis* / Q000628:therapy*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D002648:Child; D003226:Congresses as Topic / Q000592:standards*; D005260:Female; D017408:Guidelines as Topic / Q000592:standards*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D020123:Sirolimus / Q000627:therapeutic use; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000628:therapy; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus
keywords: mTOR inhibitor; subependymal giant cell astrocytoma; surgery; tuberous sclerosis complex