title: Pilomyxoid astrocytoma of the cerebellum with Williams syndrome: a case report.
abstract: METHODS
  A 3-year-old boy with Williams syndrome associated with supravalvular aortic stenosis was admitted to our hospital with disturbance of consciousness and a 2-month history of truncal ataxia. T1-weighted magnetic resonance imaging with contrast medium showed a heterogeneously enhanced tumor in the right cerebellum with severe hydrocephalus. The patient underwent tumor resection via suboccipital craniotomy. At the end of resection of the tumor, sudden cardiac arrest occurred after ST segment elevation. Despite immediate cardiopulmonary resuscitation, the patient died. Histological examination of the cerebellar tumor revealed that the tumor consisted of monomorphous bipolar spindle cells on a background of myxoid matrix, and angiocentric arrangement without Rosenthal fibers or eosinophilic granular body. The final diagnosis was pilomyxoid astrocytoma.
  
  
  CONCLUSIONS
  This case of Williams syndrome with cerebellar pilomyxoid astrocytoma suggests the importance of investigation of the development of brain tumors and occurrence of intraoperative cardiac arrest associated with Williams syndrome.
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Chonan
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kanamori
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan. kuma@nsg.med.tohoku.ac.jp.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Kumabe
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Ryuta
authors.initials: R
authors.lastname: Saito
authors.affiliation: Department of Pathology, Tohoku University Hospital, Sendai, Japan.
authors.forename: Mika
authors.initials: M
authors.lastname: Watanabe
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Teiji
authors.initials: T
authors.lastname: Tominaga
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000150:complications; D001254:Astrocytoma / Q000150:complications / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D002675:Child, Preschool; D003399:Craniotomy; D016757:Death, Sudden, Cardiac; D017809:Fatal Outcome; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology; D007431:Intraoperative Complications; D008279:Magnetic Resonance Imaging; D008297:Male; D018193:Neoplasms, Complex and Mixed / Q000150:complications / Q000473:pathology* / Q000601:surgery; D018980:Williams Syndrome / Q000150:complications
keywords: Cerebellum; Pilomyxoid astrocytoma; Sudden cardiac arrest; Williams syndrome