Choroid plexus tumours

Choroid plexus tumours (CPT) are relatively rare and include three pathological subtypes: papilloma (CPP) (benign); carcinoma (CPC) (malignant); atypical papilloma (ACPP) (intermediate). Most cases of CPT occur during early childhood and infancy; however, occurrence at all paediatric ages as well as adulthood has been documented. The main treatment of CPT is surgical, aiming for complete resection in all subtypes of CPT. The location and vascularity of CPT present a special surgical challenge. Surgical excision of CPP can be curative. In the malignant CPC form, adjuvant chemotherapy is indicated. The role of radiotherapy is controversial. A subset of patients with CPT harbour germline or somatic TP53 mutations characterized by greater tumour aggressiveness and decreased survival.