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Choroid plexus tumours 

Choroid plexus tumours
Chapter:
Choroid plexus tumours
Author(s):

Maria Santos

, Eric Bouffet

, Carolyn Freeman

, and Mark M. Souweidane

DOI:
10.1093/med/9780199651870.003.0006
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date: 02 December 2020

Choroid plexus tumours are rare, intraventricular, primary central nervous system tumours derived from the choroid plexus epithelium. They occur predominantly in children and are classified based on histological criteria as choroid plexus papilloma, atypical choroid plexus papilloma, and choroid plexus carcinoma. Choroid plexus carcinomas can occur in the context of Li–Fraumeni syndrome, where the TP53 germline mutation predisposes patients to a wide range of neoplasms. Treatment of these tumours is challenging, due to their high vascularity and the young age of the patients. While surgery is the mainstay of treatment of all choroid plexus tumours, the exact role of adjuvant therapy, particularly in choroid plexus carcinoma, is still unclear. For incompletely resected tumours, there is evidence that neoadjuvant chemotherapy can facilitate second-look surgery and reduce the risk of intraoperative bleeding. However, the role of adjuvant radiation after complete resection remains unclear.

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