Cite

Copy

Tap on and choose 'Add to Home Screen' to create a shortcut app

Tap on and choose 'Add to Home Screen/Install App' to create a shortcut app

Epidemiology of Supratentorial Choroid Plexus Tumors in Children

This page was last updated on May 9th, 2017

Incidence and Prevalence

  • 0.3/100,000: The average annual incidence of choroid plexus tumors is 0.3/100,000 (103, 104).
  • More common in children: Choroid plexus tumors account for 0.4–0.6% of all CNS tumors, but 4% of pediatric brain tumors (2, 21, 25, 50, 72).

Choroid plexus papilloma

  • 80–90% of choroid plexus tumors: More frequent than choroid plexus carcinomas, choroid plexus papillomas account for 80–90% of choroid plexus tumors (58).
  • 8% congenital: Congenital choroid plexus papillomas exist and account for 8% of brain tumors diagnosed prenatally (59, 72).

Choroid plexus carcinoma

  • Rare: Choroid plexus carcinomas account for 10–20% of choroid plexus tumors (8, 58).

Choroid plexus adenoma

  • Extremely rare: Only 4 pediatric cases of choroid plexus adenoma have been reported (3, 82).

Choroid plexus cysts

  • Relatively common (60).
  • 1% of fetuses: Choroid plexus cysts can be present in 1% of fetuses in utero and often decrease before birth (58). They are particularly prevalent in fetuses with trisomy 18/21 and Aicardi syndrome.

Age Distribution

  • Mean age of diagnosis 10 months: 40% of choroid plexus tumors are diagnosed by the age of 1 year, and 70–75% occur before the age of 2 years (23).

Choroid plexus papilloma

  • Presents in children younger than 5 years. Median age at diagnosis in one study was 18 -55 months, and 8 months for atypical choroid plexus papilloma (Koh 2014).

Choroid plexus carcinoma

  • Mean age of 2 years at diagnosis (63). Median age at diagnosis in a recent study was 15 momonths (Koh 2014).

Sex Predilection

  • Male: There is an increased incidence of choroid plexus tumors in males versus females (50, 58).

Geographic Distribution

  • None

Risk Factors

  • None

Relationships to Other Disease States and Syndromes

Choroid plexus tumors have been found in other diseases with chromosomal imbalance and sometimes are associated with the following diseases:

  • Li-Fraumeni syndrome: Choroid plexus tumors have been associated with mutations or deletions of p53 (3, 76, 95).  Animal models of choroid plexus carcinoma can be induced by SV-40,  causing a modification of the His-1 transcript. Therefore His-1 might be a potential target (53).
  • NF 2 (54).
  • Aicardi syndrome (9, 38, 84, 85).
  • Down syndrome (40).
  • von Hippel-Lindau disease (11, 12).
  • Sturge-Weber syndrome (1).
  • TP53: A mutation of the TP53 germline was detected in familial cases but not in sporadic choroid plexus tumors (5, 61).

Your donations keep us going

The ISPN Guide is free to use, but we rely on donations to fund our ongoing work and to maintain more than a thousand pages of information created to disseminate the most up-to-date knowledge in the field of paediatric neurosurgery.

By making a donation to The ISPN Guide you are also indirectly helping the many thousands of children around the world whose treatment depends on well-informed surgeons.

Please consider making a donation today.

Use the app

The ISPN Guide can be used as a standalone app, both on mobile devices and desktop computers. It’s quick and easy to use.

Fully featured

Free registration grants you full access to The Guide and host of featured designed to help further your own education.

Stay updated

The ISPN Guide continues to expand both in breadth and depth. Join our mailing list to stay up-to-date with our progress.