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Pilomyxoid astrocytoma of the cerebellum with Williams syndrome: a case report

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Abstract

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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Acknowledgments

The authors thank Dr. Toshiaki Hayashi, Dr. Tomomi Kimiwada, Dr. Reizo Shirane, at Department of Neurosurgery, Miyagi Children’s Hospital, Dr. Syuhei Kakizaki at Department of Pediatrics, Tohoku University Graduate School of Medicine, Dr. Daizo Sato at Department of Anesthesiology, Tohoku University School of Medicine, Dr. Yoshiko Tanaka at Department of Pediatrics, Sendai Red Cross Hospital, Ritsuko Takahashi at Department of Neonatal Intensive Care Unit, Perinatal Medical Center, Sendai Red Cross Hospital, Takashi Tanaka and Ozawa Akira at Department of Pediatric Cardiology, and Miyagi Children’s Hospital for kindly providing the data and helpful discussion.

Conflict of interest

The authors have no personal financial or institutional interest in any of the drugs, materials, or devices in the article.

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Correspondence to Toshihiro Kumabe.

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Chonan, M., Kanamori, M., Kumabe, T. et al. Pilomyxoid astrocytoma of the cerebellum with Williams syndrome: a case report. Childs Nerv Syst 29, 1211–1214 (2013). https://doi.org/10.1007/s00381-013-2107-7

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  • DOI: https://doi.org/10.1007/s00381-013-2107-7

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