Fact-sheet: Choroid plexus papilloma


Updated on 09/17/2021 at 12:16 PM

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Definition

Intraventricular papillary tumors derived from the choroid plexus:
-choroid plexus papilloma (WHO grade I)
-choroid plexus carcinoma (WHO grade III)
Epidemiology
-2-4% of all pediatric brain tumors (86% present <5 years).
-0.5% of adult brain tumors.
Location:
-50% atrium of the lateral ventricles (left > right).
-40% fourth ventricle - foramen of Luschka.
-10% third ventricle.

Clinical features

-Increased head circumference in infants – macrocrania.
-Increased intracranial pressure, bulging fontanelles, headache, vomiting.
-Focal neurological deficits (more suggestive of an invasive form – carcinoma)

Ultrasound

-Hyperechoic intraventricular mass, isoechoic to normal choroid plexus.
-Ventricular dilatation > hydrocephalus.
-Color and pulsed Doppler: hypervascularized mass with an arterial pedicle.

CT

-Lobulated intraventricular mass with a "cauliflower-like" appearance -iso- or hyperdense on unenhanced CT (75%) -intense enhancement after iodinated contrast administration. -Numerous intralesional calcifications. -Hydrocephalus - transependymal CSF resorption. -Heterogeneous enhancement and infiltration of adjacent brain parenchyma suggest an infiltrative form (carcinoma).

MRI

-T1: well-defined lobulated mass, iso- to hypointense.
-T2: well-defined lobulated mass, iso- to hyperintense with flow voids.
-FLAIR: hydrocephalus - transependymal CSF resorption.
-T2* GRE: hypointense intralesional hemorrhagic changes and calcification.
-T1 C+: intense and homogeneous enhancement. Possible cysts and small foci of necrosis.

Management

Complete surgical resection.
+/- Radiotherapy-chemotherapy in infiltrative forms.

Differential diagnosis

-Choroid plexus carcinoma +++: Difficult to distinguish on imaging.
Suggested by: focal neurologic deficits, more heterogeneous enhancement, infiltration of adjacent brain parenchyma.
-Ependymoma: fourth ventricle – in children.
-Metastases – secondary lesions: clinical context, rare in children.
-Vascular lesions: cavernous angioma.
-Xanthogranulomatous cystic degeneration.