Choroid Plexus Papillomas in two siblings: Case report
Date
2009Source Title
Turkish Neurosurgery
Print ISSN
1019-5149
Volume
19
Issue
3
Pages
281 - 284
Language
Turkish
Type
ArticleItem Usage Stats
109
views
views
39
downloads
downloads
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 plexusFamiliar
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