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Pathology of Choroid Plexus Neoplasms

Overview

Choroid plexus neoplasms are rare, intraventricular, primary central nervous system (CNS) tumors derived from choroid plexus epithelium that are seen predominantly in children. Choroid plexus tumors are graded based on the World Health Organization (WHO) classification scheme and include choroid plexus papilloma (CPP) (WHO grade I) (see the following image), atypical choroid plexus papilloma (WHO grade II), and choroid plexus carcinoma (CPC) (WHO grade III).

This coronal T1-weighted magnetic resonance image

This coronal T1-weighted magnetic resonance image (MRI) following contrast administration shows a homogeneously enhancing choroid plexus papilloma within the right lateral ventricle of a 1-year-old boy.

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The overall annual incidence of choroid plexus neoplasms for all ages is 0.3 cases per million. Although these tumors arise in all age groups, their peak incidence is within the mid-teenage years.
In adults, they account for less than 1% of primary intracranial neoplasms, whereas choroid plexus tumors represent up to 5% of pediatric brain tumors, and up to 20% of those arising in children aged 1 year and younger.
Occasional fetal and congenital examples have been presented.

Irrespective of patient age, choroid plexus papillomas outnumber choroid plexus carcinomas by a 5:1 ratio. Up to 90% of choroid plexus tumors in children are papillomas, and up to 70% of all choroid plexus papillomas occur in children younger than 2 years. Choroid plexus carcinomas are also far more common in the pediatric population, with approximately 80% of choroid plexus carcinomas occurring in children. There is no significant sex predilection.

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