Choroid Plexus Papilloma
- Benign, lobulated, intraventricular mass arising from choroid plexus epithelium
- May disseminate via cerebrospinal fluid (CSF)
- Fibrovascular connective tissue covered by cuboidal or columnar choroid plexus epithelium
- WHO grade I (typical choroid plexus papilloma [CPP]) or II (atypical CPP)
- Cysts and hemorrhage may be seen
- Rare malignant transformation
- Rare necrosis, invasion of adjacent brain parenchyma
- Invasion suggests choroid plexus carcinoma
- Association with Li-Fraumeni and Aicardi syndromes
- Association with simian virus 40 (SV40) infection
- Usually arising in lateral or fouth ventricles
- Lateral ventricle: majority of patients are <20 years old; no gender predilection
- Fouth ventricle: adults are more commonly affected; slight male gender predilection
- Presenting signs/symptoms often related to increased intracranial pressure secondary to either CSF overproduction or obstruction/impaired CSF resorption (communicating hydrocephalus)
- Macrocephaly, bulging fontanelles
- Nausea, vomiting, headaches, ataxia
- Gross-total resection
- Recurrence rare
- Excellent prognosis: 5-year survival rate, nearly 100%
- Lobulated, frond-like, avidly enhancing intraventricular mass
- Arising in regions of choroid plexus
- Lateral ventricle atrium or trigone > foramen of Luschka or posterior medullary velum of fourth ventricle > third ventricular roof
- Hemorrhage and calcification common
- Isodense to hyperdense
- ±Calcification, hydrocephalus
- Avid homogenous enhancement on contrast-enhanced CT
- T1WI: isointense to hypointense
- T2WI: isointense to hyperintense; ±flow voids
- FLAIR: hyperintense periventricular signal secondary to transependymal CSF flow/interstitial edema from hydrocephalus
- T2*/GRE/SWI: black signal blooming secondary to calcification or hemosiderin deposition
- T1WI+C: avid homogeneous enhancement most common
- MRA: may see flow-related signal within the tumor
- MRS: elevated Cho, absent NAA, lactate peak if necrotic, elevated myoinositol may differentiate from choroid plexus carcinoma
- MRI with contrast, include both brain and spine due to risk of CSF dissemination
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
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