Choroid Plexus Papilloma (CPP)
Last Updated: March 27, 2020
- Benign, lobulated, intraventricular mass arising from choroid plexus epithelium
- May disseminate via CSF
- Fibrovascular connective tissue covered by cuboidal or columnar choroid plexus epithelium
- WHO grade I (typical 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 4th ventricles
- Lateral ventricle: majority of cases <20 years old, no gender predilection
- 4th ventricle: adults 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 nearly 100%
- Lobulated, frond-like, and avidly-enhancing intraventricular mass
- Arising in regions of choroid plexus
- Lateral ventricle atrium or trigone > foramen of Luschka or posterior medullary velum of 4th ventricle > 3rd ventricular roof
- Hemorrhage and calcification common
- Iso- to hyperdense
- ± Calcification, hydrocephalus
- Avid, homogenous enhancement on contrast-enhanced CT
- T1WI: Iso- to hypointense
- T2WI: iso- 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
- Magnetic resonance angiogram (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
Buckle C, et al. Choroid plexus papilloma of the third ventricle. Pediatr Radiol. 2007;37:725.
Naeini RM, et al. Spectrum of choroid plexus lesions in children. AJR Am J Roentgenol. 2009;192:32-40.
Osborn AG, Salzman, KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.
Safaee M, et al. Surgical outcomes in choroid plexus papillomas: an institutional experience. J Neurooncol. 2013;113:117-125.
Smith A, Smirniotopoulos J, Horkanyne-Szakaly I. From the Radiologic Pathology Archives: Intraventricular Neoplasms: Radiologic-Pathologic Correlation. Radiographics. 2013;33: 21-43.
Zhang TJ, Yue Q, Lui S, et al. MRI findings of choroid plexus tumors in the cerebellum. Clin Imaging. 2011;35: 64-67.
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