Epidemiology of Supratentorial Choroid Plexus Tumors in Children

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).