Abstract
Choroid plexus tumors (CPTs) are rare tumors of childhood and include choroid plexus papillomas (CPPs), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). CPTs often have mutations in TP53 and CPC is seen in patients with Li-Fraumeni syndrome. CPTs have relatively common characteristics on MRI. Management is primarily surgical for CPP, with chemotherapy and radiation generally reserved for CPTs that recur, or aCPP and CPC. Outcomes for CPP are very good with nearly all children cured with surgery. In contrast, CPC survival at 5 years ranges from 30 to 60%. Current studies are examining novel chemotherapy regimens.
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Liu, A.K., Hankinson, T., Wolff, J.E.A. (2018). Choroid Plexus Tumors. In: Mahajan, A., Paulino, A. (eds) Radiation Oncology for Pediatric CNS Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-55430-3_19
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