CHOROID PLEXUS TUMOURS
Choroid plexus neoplasms are rare (0.5 % of all brain tumors) and are most frequent in infancy. The majority of choroid plexus tumors are benign papillomas, but up to 30 % are classified as carcinomas.
Childhood choroid plexus tumors in sibling pairs have been reported and autosomal recessive inheritance suggested (Zwetsloot et al. 1991). Tumors of the choroid plexus have been reported in the X-linked disorder Aicardi syndrome (Robinow et al. 1986). Germline TP53 mutations are relatively frequent in children with choroid plexus tumors (Gozali et al. 2012). Though the family history may be suggestive of Li–Fraumeni syndrome in many cases, in others there may be no family history of cancer (Krutilkova et al.
2005; Tabori et al. 2010). The germline founder in most children who develop choroid plexus carcinomas (Custodio et al. 2011).
Choroid plexus angiomas were reported in two out of four patients with Perlman syndrome reported by Henneveld et al. (1999).
Choroid plexus tumors should be differentiated TP53 mutation R337H occurs at high frequency in Brazil and can be detected from endolymphatic sac tumors, which are a feature of von Hippel–Lindau disease.