title: Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients.
abstract: BACKGROUND
  Subependymal giant cell astrocytoma is a brain tumor associated with tuberous sclerosis complex. There are two treatment options for subependymal giant cell astrocytomas: surgery or mammalian target of rapamycin inhibitor. The analysis of outcome of subependymal giant cell astrocytoma surgery may help characterize the patients who may benefit from pharmacotherapy.
  
  
  METHODS
  Sixty-four subependymal giant cell astrocytoma surgeries in 57 tuberous sclerosis complex patients with at least a 12-month follow-up were included in the study. The tumor size, age of the patients, mutation in the TSC1 or TSC2 gene, indication for the surgery, and postsurgical complications were analyzed.
  
  
  RESULTS
  The mean age of patients at surgery was 9.7 years. Mean follow-up after surgery was 63.7 months. Thirty-seven (57.8%) tumors were symptomatic and 27 (42.2%) were asymptomatic. Patients with TSC2 mutations developed subependymal giant cell astrocytoma at a significantly younger age than individuals with TSC1 mutations. Four patients (6.2% of all surgeries) died after surgery. Surgery-related complications were reported in 0%, 46%, 83%, 81%, and 67% of patients with tumors <2 cm, between 2 and 3 cm, between 3 and 4 cm, >4 cm, and bilateral subependymal giant cell astrocytomas, respectively, and were most common in children younger than 3 years of age. The most common complications included hemiparesis, hydrocephalus, hematoma, and cognitive decline.
  
  
  CONCLUSIONS
  Our study indicates that subependymal giant cell astrocytoma surgery is associated with significant risk in individuals with bilateral subependymal giant cell astrocytomas, tumors bigger than 2 cm, and in children younger than 3 years of age. Therefore, tuberous sclerosis complex patients should be thoroughly screened for subependymal giant cell astrocytoma growth, and early treatment should be considered in selected patients.
authors: Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland; Department of Science, The Children's Memorial Health Institute, Warsaw, Poland. Electronic address: k.kotulska@czd.pl.
authors: Katarzyna
authors: K
authors: Kotulska
authors: Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Julita
authors: J
authors: Borkowska
authors: Department of Neurosurgery, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Marcin
authors: M
authors: Roszkowski
authors: Department of Pediatric Neurosurgery, Silesian Medical University, Katowice, Poland.
authors: Marek
authors: M
authors: Mandera
authors: Department of Neurosurgery, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Paweł
authors: P
authors: Daszkiewicz
authors: Department of Neurosurgery, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Krzysztof
authors: K
authors: Drabik
authors: Department of Radiology, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Elzbieta
authors: E
authors: Jurkiewicz
authors: Department of Physiology, Silesian Medical University, Katowice, Poland.
authors: Magdalena
authors: M
authors: Larysz-Brysz
authors: Department of Radiology, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Katarzyna
authors: K
authors: Nowak
authors: Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Wiesława
authors: W
authors: Grajkowska
authors: Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Dorota
authors: D
authors: Domańska-Pakieła
authors: Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland.
authors: Sergiusz
authors: S
authors: Jóźwiak
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001254:Astrocytoma / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D001921:Brain / Q000473:pathology / Q000601:surgery; D001932:Brain Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009154:Mutation; D019635:Neurosurgical Procedures / Q000009:adverse effects; D012307:Risk Factors; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins / Q000235:genetics; D055815:Young Adult
chemical_list: C000624650:TSC1 protein, human; C000624653:TSC2 protein, human; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins
keywords: mTOR inhibitors; outcome; prognostic factor; subependymal giant cell astrocytoma; surgery; tuberous sclerosis complex