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      • Department of Molecular Biology and Genetics
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      Choroid Plexus Papillomas in two siblings: Case report

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      Author(s)
      Okay, O.
      Dağlıoğlu, E.
      Yakıcıer, Cengiz
      Üren, Deniz
      Dalgıç, A.
      Ergüngör, F.
      Date
      2009
      Source Title
      Turkish Neurosurgery
      Print ISSN
      1019-5149
      Volume
      19
      Issue
      3
      Pages
      281 - 284
      Language
      Turkish
      Type
      Article
      Item Usage Stats
      109
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      39
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      Abstract
      Choroid plexus papilloma (CPP) is a rare, benign epithelial brain tumor of the nervous system seen particularly in infants. Familial cases are extremely uncommon. Some other form of malignant tumors was noted in the relatives of patients with CPPs, and some genetic defects regarding this coincidence were reported in the literature. These neoplasms are occasionally bilateral and hydrocephalus is an associated sign in most of the cases. We report three lateral ventricle CPPs in two siblings, at the age of 7 month and 2 years respectively. All tumors were resected with parietotemporal craniotomy and a superior temporal sulcus approach to the lateral ventricle. To avoid a concomitant need of ventriculoperitoneal shunt insertion, external ventricular drainage was inserted for a week in the postoperative period relieving symptoms of hydrocephalus. Search for a hereditary defect in the p53 gene of the second infant (7 months old) revealed no mutation. Postoperative courses were uneventful and the patients were followed for three years without any recurrence. Bilateral CPPS are rare and unusual in two siblings. A genetic predisposition such as the p53 mutation should be investigated in bilateral CPPs in particular.
      Keywords
      Choroid plexus
      Familiar
      Hydrocephalus
      Lateral Ventricle
      p53-Mutation
      Papilloma
      Protein p53
      Brain ventricle peritoneum shunt
      Case report
      Choroid plexus papilloma
      Genetics
      Hydrocephalus
      Llateral brain ventricle
      Nuclear magnetic resonance imaging
      Nucleotide sequence
      Pathology
      Preschool child
      Sibling
      Base Sequence
      Humans
      Hydrocephalus
      Infant
      Lateral Ventricles
      Magnetic Resonance Imaging
      Male
      Molecular Sequence Data
      Papilloma, Choroid Plexus
      Siblings
      Tumor Suppressor Protein p53
      Ventriculoperitoneal Shunt
      Permalink
      http://hdl.handle.net/11693/22540
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