title: Angiocentric glioma: a clinicopathologic review of 5 tumors with identification of associated cortical dysplasia.
abstract: BACKGROUND
  Angiocentric glioma is a rare, epilepsy-associated, low-grade neoplasm with a characteristic perivascular growth pattern.
  
  
  OBJECTIVE
  To describe the clinicopathologic features of 5 angiocentric gliomas and to evaluate for coexistent malformation of cortical development/cortical dysplasia.
  
  
  METHODS
  Retrospective review of the clinicopathologic features of 5 angiocentric gliomas (3 males and 2 females; median age at surgery, 10 years; range, 3-19 years).
  
  
  RESULTS
  Seizures were the most common presenting symptom (n = 4); 1 patient presented with headaches. Four of the tumors were located in the parieto-occipital, parietal, or temporal cortex and 1 case arose in the thalamus. All tumors consisted of an angiocentric growth pattern of bipolar spindle cells with mild pleomorphism. Three tumors also demonstrated a focal solid growth pattern. Evidence of adjacent malformation of cortical development/focal cortical dysplasia was observed in 4 of 4 cases with sufficient tissue for evaluation; all were Palmini et al type I lesions (type IA, n = 1; type IB, n = 3). All patients were alive at last known follow-up (17-131 months).
  
  
  CONCLUSIONS
  The thalamic location of 1 tumor represents an undescribed location for this typically superficial cortical tumor. A subset of angiocentric gliomas, similar to other low-grade chronic epilepsy-related tumors of childhood, are associated with coexistent malformation of cortical development, suggesting a developmental basis to their origin.
authors.affiliation: Department of Pathology, Cleveland Clinic, Ohio 44195, USA. marburt@ccf.org
authors.forename: Trent
authors.initials: T
authors.lastname: Marburger
authors.forename: Richard
authors.initials: R
authors.lastname: Prayson
mesh_terms: D000293:Adolescent; D001927:Brain Diseases / Q000150:complications / Q000473:pathology*; D001932:Brain Neoplasms / Q000150:complications / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000209:etiology / Q000473:pathology*; D005260:Female; D005910:Glioma / Q000150:complications / Q000473:pathology*; D006801:Humans; D008297:Male; D054220:Malformations of Cortical Development / Q000150:complications / Q000473:pathology*; D065703:Malformations of Cortical Development, Group I; D012189:Retrospective Studies; D055815:Young Adult