# Choroid plexus carcinoma

Source: https://onco.cc/cancers/choroid-plexus-carcinoma/  
OnCo record `choroid-plexus-carcinoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Choroid plexus carcinoma is a rare, aggressive brain tumour of infants and young children that grows from the tissue that makes spinal fluid inside the brain's ventricles, causing fluid build-up and pressure. It is strongly linked to an inherited TP53 fault (Li-Fraumeni syndrome), so families are tested; treatment is surgery, then chemotherapy and, in older children, radiotherapy.

## Summary

Choroid plexus carcinoma is the grade 3 member of the choroid plexus tumours in the WHO central nervous system classification, distinguished from choroid plexus papilloma and atypical papilloma by frequent mitoses, necrosis and invasion (NCI PDQ). Choroid plexus tumours make up about 2 to 5 percent of paediatric brain tumours and most present with severe hydrocephalus; in 39 patients (31 papillomas, 8 carcinomas) perioperative management and oncological care remained debated (J Neurosurg Pediatrics 2012). Of 42 choroid plexus tumour patients at Children's Hospital Los Angeles, 6 (16.7 percent) had features of Li-Fraumeni syndrome (Pediatric Blood and Cancer 2012), and in southern Brazil, where the low-penetrance TP53 R337H founder mutation is common, 9 of 13 children with choroid plexus carcinoma (69 percent) carried it (Cancer 2011).

How it differs from its parent: a tumour of infancy arising inside the ventricles with hydrocephalus as the presenting problem, extreme vascularity that makes surgery hazardous, and the strongest association with germline TP53 mutation of any childhood brain tumour, so that germline testing is part of the work-up.

How common: a minority of the 2 to 5 percent of paediatric brain tumours that are choroid plexus tumours (J Neurosurg Pediatrics 2012).

Treatment: maximal safe resection, often in stages after chemotherapy to shrink and devascularise the tumour, then platinum- and etoposide-based chemotherapy with radiotherapy for older children and residual disease, as the PDQ childhood brain tumour summaries describe; TP53 carriers are treated with radiotherapy sparing where possible because of second cancers.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Choroid plexus carcinoma (WHO grade 3); Malignant choroid plexus tumour; CPC
- Tags: subtype-page; wave4; rare
- Group: paediatric
- Burden: Choroid plexus tumours are about 2 to 5 percent of paediatric brain tumours, and carcinomas the minority: 8 carcinomas among 39 choroid plexus tumours in one 20-year series (J Neurosurg Pediatrics 2012). No registry figure for the carcinoma alone is in the sources read.
- Biomarkers: Germline TP53 mutation (Li-Fraumeni syndrome); Mitotic count, necrosis and brain invasion (grade 3 criteria); Extent of resection; Hydrocephalus at presentation

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/choroid-plexus-carcinoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/choroid-plexus-carcinoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/choroid-plexus-carcinoma/#what-it-is
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/choroid-plexus-carcinoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/choroid-plexus-carcinoma/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/choroid-plexus-carcinoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/choroid-plexus-carcinoma/#science [2 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/choroid-plexus-carcinoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/choroid-plexus-carcinoma/#living-with-it [7 questions, 3 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/choroid-plexus-carcinoma/coming/ [4 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/choroid-plexus-carcinoma/data/ [15 connected records]

## Standard of care

- All cases: Maximal safe resection, staged after chemotherapy where the tumour is too vascular; platinum- and etoposide-based chemotherapy; radiotherapy for older children and residual disease; germline TP53 testing. ([Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/), [Li-Fraumeni syndrome (germline TP53)](https://onco.cc/terms/li-fraumeni/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Choroid_plexus_carcinoma
- NCI PDQ: rare cancers of childhood: https://www.cancer.gov/types/childhood-cancers/patient/rare-childhood-cancers-pdq
- J Neurosurg Pediatrics 2012: treatment of choroid plexus tumours, 20-year single-institution experience: https://doi.org/10.3171/2012.8.peds12132
- Pediatric Blood and Cancer 2012: choroid plexus tumours and Li-Fraumeni syndrome, Children's Hospital Los Angeles 1991 to 2010: https://doi.org/10.1002/pbc.23349
- Cancer 2011: TP53 R337H and paediatric choroid plexus carcinoma in southeast Brazil: https://doi.org/10.1002/cncr.25826

## Connected records

- cancers: [Adrenocortical carcinoma](https://onco.cc/cancers/adrenocortical/), [Atypical teratoid/rhabdoid tumour (ATRT)](https://onco.cc/cancers/atrt/), [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Childhood cancers (all types)](https://onco.cc/cancers/childhood-cancers/), [Pineoblastoma](https://onco.cc/cancers/pineoblastoma/), [Pineocytoma and pineal parenchymal tumour of intermediate differentiation](https://onco.cc/cancers/pineal-parenchymal-tumours/)
- technologies: [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/)
- targets: [TP53](https://onco.cc/targets/tp53/)
- drugs: [Carboplatin](https://onco.cc/drugs/carboplatin/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Etoposide](https://onco.cc/drugs/etoposide/)
- terms: [Li-Fraumeni syndrome (germline TP53)](https://onco.cc/terms/li-fraumeni/)

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JSON: https://onco.cc/api/v1/entities/choroid-plexus-carcinoma.json