Monday, July 6, 2009

6-27-2009 Case 1 Answer - Choroid Plexus Carcinoma



CT - demonstrates a hyperdense structure in the lateral ventricle causing hydrocephalus in a young patient. Pathology revealed a choroid plexus carcinoma.

  • Neoplasms of ventricular system are usually easy to recognize but pathology is sometimes difficult to determine by imaging characteristics
  • Choroid plexus - found in the ventricles - highly vascular and produces CSF
  • Choroid plexus neoplasms - usually highly vascular and produce hydrocephalus
  • Benign - papilloma; Malignant - carcinoma
  • Most frequently found in atria and posterior portion of lateral ventricles
  • Other highly vascular tumors are commonly seen in this site - mets and meningioma
  • Ependymal cells - ependymoma
  • Subependymal glial cells - subependymomas and subependymal giant cell astrocytoma
  • Septum pellucidum - central neurocytoma
  • Choroid plexus - found most commonly in atria, third ventricle and 4th ventricle (not seen in temporal horns or aqueduct of Sylvius)
  • 50% of tumors are in lateral ventricles, 40% in 4th and 5% in 3rd.
  • Rare - extraventricular areas like CPA, suprasellar cistern etc
  • Choroid plexus tumors aren't that common overall but 10-20% of brain tumors in those less than 1 yr.
  • 80% are benign papilloma and rest are carcinoma
  • Those in lateral ventricle are most likely to present <10
  • Most present due to symptoms of hydrocephalus
  • Other symptoms - cranial nerve palsies, seizures, coma, and one report of psychosis
  • Choroid plexus tumors - have an association with Li-Fraumeni and Aicardi syndromes
  • Choroid plexus papilloma - 5 yr survival - practically 100%
  • Choroid plexus carcinoma - 5 yr survival - 26-50%
  • Choroid plexus tumors - cauliflower like well circumscribed masses with lobulations
  • hemorrhage and cyst formation can be seen
  • Signs of carcinoma - necrosis and parenchymal invasion
  • Lateral ventricle choroid plexus - attached at trigone
  • 3rd ventricle - attached at roof
  • 4th ventricle - attached at posterior medullary velum
  • Can have ball-valve effect for hydrocephalus
  • Atypical choroid plexus papilloma - one or 2 histologic malignant features but not enough for carcinoma
  • Papilloma and Carcinoma (but much more frequent for carcinoma) can have seeding of CSF
  • CT - most tumors are iso or hyperattenuating
  • CT - with enhancement - avid enhancement
  • CT - 24% see calcifications
  • T1 MR - iso or hypo to brain
  • T2 - variable and often see flow voids within the lesion
  • T1 postcon - avid enhancement
  • Carcinoma - may have more variable CT and MR appearance; may have less hydrocephalus; look for parenchymal invasion and vasogenic edema
  • Carcinoma - Increased uptake on FDG PET
  • Imaging - papilloma and carcinoma has a lot of overlap therefore unless obvious malignant features are present cannot exclude carcinoma when imaging looks like a papilloma
  • Lateral ventricular lesions - supplied by anterior choroidal, medial posterior choroidal and lateral posterior choroidal arteries
  • 4th ventricular - choroidal branches of PICA
  • Lesions in trigone - less than 10 - think papilloma/carcinoma; 10-40 - think low grade glial like ependymoma or subependymoma; >/= 40 - think meningioma, mets, lymphoma
  • Intraventricular meningioma - rare and almost always older (caveat - 1/5 of all meningiomas in children are intraventrciular)

References - http://radiographics.rsnajnls.org/cgi/content/full/22/6/1473 and http://www.ajnr.org/cgi/content/full/20/5/882

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