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J Neurosurg Pediatr. 2013 Feb;11(2):115-8. doi: 10.3171/2012.10.PEDS12219. Epub 2012 Nov 23.

Choroid plexus papilloma and Pierpont syndrome.

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Division of Pediatric Neurosurgery, Cohen Children’s Medical Center and the Hofstra North Shore–LIJ School of Medicine, New Hyde Park, New York 11030, USA.


The authors describe the case of a child who presented with hydrocephalus and phenotypic features characteristic of a multiple congenital anomalies/mental retardation syndrome. Dysmorphic facies, medial plantar lipomatosis, and developmental delay were observed in this case and are identical to documented findings of Pierpont syndrome diagnosed in 3 boys. This is the fourth case reported to date and is the first documented case of an oncological process- an intraventricular atypical choroid plexus papilloma tumor-found in association with Pierpont syndrome. Syndromes associated with choroid plexus papilloma are reviewed.

[Indexed for MEDLINE]

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