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Choroid Plexus Papilloma (CPP)

Last Updated: March 27, 2020

Open Table of Contents: Choroid Plexus Papilloma (CPP)

Figure 1: Sagittal FLAIR (top row left) and T1WI post contrast (top row right) images demonstrate a lobular, avidly enhancing lesion within the third ventricle. T2 coronal (bottom) image illustrates dilatation of the foramen of Monro and of the lateral ventricles due to obstruction by this choroid plexus papilloma.

Figure 2: This lobulated enhancing intraventricular lesion is of intermediate signal on axial FLAIR (top row left) image. The axial T2WI (top row right) demonstrates surrounding CSF signal clefts that more easily characterize this lesion as intraventricular. This choroid plexus papilloma also demonstrates avid enhancement on T1WI post contrast (bottom row left) and hyperperfusion on cerebral blood volume image (bottom row right).

Basic Description

  • Benign, lobulated, intraventricular mass arising from choroid plexus epithelium
  • May disseminate via CSF

Pathology

  • 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

Clinical Features

  • 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
  • Treatment

    • Gross total resection
    • Recurrence rare
  • Excellent prognosis: 5-year survival nearly 100%

Imaging Features

  • General
    • 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
  • CT

    • Iso- to hyperdense
    • ± Calcification, hydrocephalus
    • Avid, homogenous enhancement on contrast-enhanced CT
  • MRI

    • 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

Imaging Recommendations

  • 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

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.07

References

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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