Case Report: 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. Conclusion: 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.
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