Choroid plexus papilloma and carcinoma
 Discussion of Choroid plexus papilloma and carcinoma
- Neoplasms of the choroid plexus arise anywhere this tissue exists.
- Lateral ventricle is the most common site (50% of cases) for these tumors, followed by the fourth ventricle (40%) and the third ventricle (5%). This correlates with the amount of choroidal tissue present.
- Nearly half of these tumors manifest in the 1st decade of life.
- Most choroid plexus tumors (about 80%) occur as the benign, slowly growing choroid plexus papilloma (WHO grade I tumor).
- The other 20% of cases manifest as a much more biologically aggressive WHO grade III tumor, the choroid plexus carcinoma.
- Tumors that arise in the lateral ventricle are much more common in patients 10 years of age or less, whereas those that arise in the fourth ventricle are fairly evenly distributed among patients 0–50 years of age.
- Choroid plexus tumors are associated with hydrocephalus and symptoms related to increased intracranial pressure.
- In most cases, the increased intraventricular pressure is secondary to an increase in the production of CSF by the tumor.
- Simple obstruction of CSF flow from large or well-placed intraventricular masses is also a contributing factor in some cases.
- The prognosis for patients with choroid plexus papilloma is excellent, with a report of 100% survival at 5 years after surgical resection in one large series, and adjuvant therapy is not indicated in these patient.
- The prognosis for patients with choroid plexus carcinoma is guarded, with an overall 5-year survival rate of 26%–50%.
- Presence of residual disease on postoperative images is an especially poor prognostic factor.
- Radiation therapy is often not an option as adjuvant therapy in these patients because of their young age.
 Imaging Findings for Choroid plexus papilloma and carcinoma
- Isoattenuated to hyperattenuated intraventricular masses without brain invasion on nonenhanced CT images
- Intense enhancement on contrast-enhanced images.
- Hydrocephalus is very common.
- Calcification is noted in 24% of choroid plexus tumors.
- Extension from one ventricle to another or into the cerebellopontine angle is a characteristic feature of choroid plexus tumors.
- On MR images, choroid plexus papillomas appear as isointense to hypointense intraventricular masses compared with normal brain parenchyma with short TR pulse sequences and variable signal intensity masses with long TR pulse sequences.
- Flow voids, consistent with flowing blood, are common.
- Follow-up postoperative studies with contrast-enhanced MR imaging of the spine is recommended by some to exclude the possibility of seeding from choroid plexus papillomas.
 Paplloma vs Carcinoma
- Choroid plexus carcinomas tend to be more heterogeneous than choroid plexus papillomas on CT and MR images.
- The findings of extraventricular extension of a choroid plexus tumor into the brain parenchyma, heterogeneity of signal intensity, and the presence of vasogenic edema in the cerebral white matter all favor the imaging diagnosis of a choroid plexus carcinoma.
Patient #1: Choroid plexus papilloma
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 References for Choroid plexus papilloma and carcinoma
- Kelly K. Koeller, and Glenn D. Sandberg. From the Archives of the AFIP: Cerebral Intraventricular Neoplasms: Radiologic-Pathologic Correlation. RadioGraphics 2002 22: 1473-1505.