Choroid plexus papilloma

Last edited and medically reviewed by Alaric Steinmetz on 19.08.2023

Synonyms

Choroid Plexus Papilloma, CPP

ICD-10

D33.0

ICD-11

2A00.22

Choroid plexus papilloma is a rare benign brain tumor (WHO Grade I) that originates from the epithelium of the choroid plexus, the Choroid plexus.

Epidemiology

Choroid plexus papillomas can occur at any age, but approximately 70 % of patients are diagnosed before the age of 2 years[^1].

Location

Choroid plexus papillomas can occur in all areas where the choroid plexus is located. However, choroid plexus papillomas preferentially occur in the Lateral ventricle.

Imaging

CT imaging

On CT, most choroid plexus papillomas appear as an isodense/hyperdense lobulated mass with homogeneous contrast enhancement. Calcifications of varying severity occur in approximately 4 - 20 % of cases[^3].

MRI imaging

The gold standard for imaging choroid plexus papillomas is MRI imaging. On MRI, choroid plexus papillomas appear as a well-defined extra-axial lesion with fern-like projections, with contrast enhancement and no invasion of the brain parenchyma. In rare cases, choroid plexus papillomas may show little or no contrast enhancement[^3].

Choroid plexus papilloma of the third ventricle on MRI — Coronal MRI image of a patient with a choroid plexus papilloma in the third ventricle.

Choroid plexus papilloma in the third ventricle — Coronal MRI image of a patient with a choroid plexus papilloma in the third ventricle.

Prognosis

Malignant transformation may occur in rare cases; in a case series from 2007, malignant transformation into a WHO Grade 3 tumor within 59 months was observed in 2 of 124 patients[^2].