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Health condition · Clinically reviewed

Choroid plexus tumours, rare intraventricular tumours from papilloma to carcinoma.

A UK patient and family guide to choroid plexus papilloma and carcinoma. Surgery, chemotherapy, proton beam therapy and Li-Fraumeni testing in one place.

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A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced from guidance

    Checked against SIOP-CPT, NICE, RCR and peer-reviewed paediatric neuro-oncology sources you can see at the end.

  • 03

    Current for 2026

    Reflects modern UK paediatric neuro-oncology practice, including proton beam therapy and TP53 germline testing.

Key facts

Choroid plexus tumours at a glance.

The essentials, in plain English. What these tumours are, who they affect, and where they are treated in the UK.

  • What it is

    A rare intraventricular tumour arising from choroid plexus epithelium, ranging from benign papilloma to aggressive carcinoma.

  • Who it affects

    Most common in infants and young children, accounting for around 15 per cent of infant brain tumours and up to 4 per cent of paediatric CNS tumours.

  • WHO grades

    Choroid plexus papilloma (grade 1, around 75 per cent), atypical papilloma (grade 2), and choroid plexus carcinoma (grade 3, around 25 per cent).

  • Genetic link

    Li-Fraumeni syndrome (germline TP53) is found in 40 to 50 per cent of children with carcinoma. Germline testing is mandatory.

  • How it presents

    Hydrocephalus from CSF overproduction and mass effect, focal deficits, seizures and, in infants, a bulging fontanelle.

  • Where it is treated

    UK specialist paediatric neuro-oncology centres, including Great Ormond Street, Alder Hey, Birmingham, Bristol and Manchester.

Why this guide matters

Rare tumours, specialist care.

Choroid plexus tumours are rare and need coordinated care at a UK paediatric neuro-oncology centre. Three principles shape everything on this page.

  • Complete resection is decisive

    Gross-total surgical resection is the single strongest predictor of outcome across all grades of choroid plexus tumour.

  • Germline testing changes care

    TP53 testing for Li-Fraumeni syndrome is mandatory in carcinoma and reshapes surveillance, family screening and radiotherapy planning.

  • Multimodal treatment for carcinoma

    Carcinoma is treated with a combination of surgery, chemotherapy and (in older children) proton beam radiotherapy through SIOP-CPT protocols.

How the diagnosis is made

From first symptoms to a clear plan.

The steps a UK paediatric team will normally follow, in order, so families know what to expect and why each step matters.

  1. 01

    Assessing

    Clinical assessment

    Head circumference, fontanelle, developmental history, cranial nerve exam and signs of raised intracranial pressure.

  2. 02

    Assessing

    MRI brain with gadolinium

    A well-enhancing intraventricular mass, often with hydrocephalus, calcification or haemorrhage on imaging.

  3. 03

    Assessing

    MRI whole spine

    Screens for leptomeningeal and spinal drop metastases, present in 20 to 40 per cent of carcinomas at diagnosis.

  4. 04

    Confirming

    Histology and molecular profiling

    Biopsy or resection tissue reviewed with IHC (transthyretin, S100, cytokeratin, INI-1) and molecular studies.

  5. 05

    Confirming

    CSF cytology

    Lumbar CSF sampled once safe (usually post-operatively) to look for tumour cells and confirm metastatic stage.

  6. 06

    Planning

    Germline TP53 testing

    Mandatory for choroid plexus carcinoma. Confirms or excludes Li-Fraumeni syndrome and triggers family cascade testing.

  7. 07

    Planning

    Specialist MDT review

    Paediatric neuro-oncology, neurosurgery, radiology, pathology and clinical genetics agree the plan at a UK specialist centre.

Typical timeline: from urgent scan to specialist MDT plan within days at a UK paediatric neuro-oncology centre.

Symptoms

What families and clinicians tend to notice.

Most symptoms come from raised intracranial pressure due to hydrocephalus. Focal signs and seizures depend on tumour location.

  • Bulging fontanelle

    In infants, a tense or bulging soft spot with a rapidly enlarging head circumference is a red flag for hydrocephalus.

  • Vomiting and irritability

    Early-morning vomiting, feeding difficulty and unusual irritability can all signal raised intracranial pressure.

  • Drowsiness and reduced feeding

    Excess sleepiness, poor feeding and failure to thrive can develop as pressure builds inside the ventricles.

  • Papilloedema in older children

    Swelling of the optic disc on fundoscopy, sometimes with blurred vision or double vision.

  • Focal neurological signs

    Weakness, gait disturbance, cranial nerve palsies or coordination problems depending on tumour location.

  • Seizures

    New-onset seizures in a young child always warrant urgent neurological assessment and brain imaging.

  • Developmental regression

    Loss of previously acquired milestones can be an early clue in infants and toddlers.

  • Red flag - rapid head growth

    A head circumference crossing centiles upward in an infant is a red flag needing urgent paediatric assessment.

Treatment

How choroid plexus tumours are treated in the UK.

Surgery first, then a tailored plan by WHO grade. Papilloma is often cured by resection alone; carcinoma needs a multimodal SIOP-CPT approach.

  • Maximum safe resection

    Gross-total resection by a specialist paediatric neurosurgeon is the single strongest prognostic factor across all grades.

  • Surgery alone for CPP

    Grade 1 papilloma is usually cured by complete resection, with imaging surveillance rather than adjuvant treatment.

  • Adjuvant chemotherapy

    For carcinoma, multi-agent chemotherapy (carboplatin, cyclophosphamide, etoposide, vincristine, methotrexate, doxorubicin) per SIOP-CPT protocols.

  • Second-look surgery

    Considered after chemotherapy to remove residual disease and improve the chance of long-term control.

  • Proton beam therapy

    Preferred for children needing radiotherapy - reduces cognitive, endocrine and secondary tumour risks compared with photon therapy.

  • Craniospinal radiotherapy

    Reserved for metastatic (M+) carcinoma in children old enough to tolerate it, typically above three years of age.

  • High-dose chemotherapy + ASCT

    Autologous stem cell rescue after high-dose chemotherapy is used selectively for progressive or relapsed carcinoma.

  • Hydrocephalus management

    External ventricular drain, ventriculoperitoneal shunt or endoscopic third ventriculostomy as needed before or after surgery.

What this guide is based on

The sources behind every claim on this page.

UK and international paediatric neuro-oncology guidance, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your paediatric neuro-oncology team knows your child and can tell you which parts apply. If in doubt, contact them directly.

  • SIOP-CPT international collaborative group. Treatment protocols for choroid plexus tumours.

  • NICE. Improving outcomes for people with brain and other CNS tumours (CSG10) and related guidance.

  • Royal College of Radiologists. Paediatric radiotherapy standards and proton beam referral pathways.

  • Villani et al. Biochemical and imaging surveillance in germline TP53 mutation carriers.

  • Chompret and updated Li-Fraumeni testing criteria.

  • The Brain Tumour Charity and Cancer Research UK patient information on paediatric brain tumours.

Red flags

When to seek urgent help.

The situations that need immediate paediatric neurosurgical or oncology input, and the long-term family concerns that also need attention.

  • Rapidly rising intracranial pressure

    Persistent vomiting, drowsiness, bulging fontanelle or reduced consciousness need emergency paediatric neurosurgical assessment.

  • New-onset focal deficit

    Sudden weakness, cranial nerve palsy or gait disturbance in a child warrants urgent brain imaging.

  • First seizure in an infant

    Any first seizure in an infant or young child needs urgent paediatric review and brain imaging.

  • Suspected spinal drop metastasis

    Back pain, leg weakness or bladder or bowel change in a child with a known CNS tumour needs urgent MRI of the whole spine.

  • Post-operative deterioration

    A sudden change in conscious level after surgery may indicate shunt failure, bleeding or CSF leak and needs immediate review.

  • Li-Fraumeni features in the family

    Early breast cancer, sarcoma, adrenocortical or brain tumours in close relatives should prompt clinical genetics referral.

  • Second primary malignancy

    Children with germline TP53 need lifelong surveillance for breast, soft tissue, adrenocortical, brain and blood cancers.

  • Radiotherapy in very young children

    Craniospinal radiotherapy under three years carries major neurocognitive risk - proton beam and chemotherapy strategies are preferred.

  • Failure to thrive

    A persistent drop in weight or feeding in an infant with neurological signs deserves urgent paediatric assessment.

Living with it

A long journey, with a team behind you.

Care for a child with a choroid plexus tumour continues long after treatment ends. Rehabilitation, surveillance and family support all matter.

A quiet reminder

You are not alone in this.

UK charities including The Brain Tumour Charity, CCLG and Young Lives vs Cancer offer practical, emotional and financial help for families.

  1. 01 Centre

    Care at a specialist unit

    Treatment belongs in a UK paediatric neuro-oncology centre with neurosurgery, oncology, radiology, pathology and genetics all under one roof.

  2. 02 Rehab

    Neuro-rehabilitation matters

    Physiotherapy, occupational therapy, speech and language and neuropsychology help children rebuild skills after surgery and treatment.

  3. 03 Surveillance

    Long-term follow-up

    Regular MRI, endocrine review and neuropsychology assessments continue for years after treatment to catch late effects early.

  4. 04 Family

    Family and genetic support

    Clinical genetics, Li-Fraumeni surveillance and charity support (The Brain Tumour Charity, CCLG, Young Lives vs Cancer) walk families through the journey.

Frequently asked

Everything families ask us about choroid plexus tumours.

Quick answers on grades, surgery, chemotherapy, proton beam therapy and TP53 testing.

  • What are choroid plexus tumours?

    Rare intraventricular brain tumours arising from choroid plexus epithelium, the tissue that produces cerebrospinal fluid. They range from benign choroid plexus papilloma (grade 1) through atypical papilloma (grade 2) to malignant choroid plexus carcinoma (grade 3).

  • How common are they?

    They make up around 1 to 4 per cent of paediatric CNS tumours overall but about 15 per cent of brain tumours in infants under one year. In children the lateral ventricles are the most common site; in adults, the fourth ventricle.

  • Why is TP53 germline testing so important?

    Around 40 to 50 per cent of children with choroid plexus carcinoma carry a germline TP53 mutation - Li-Fraumeni syndrome. Confirming this changes surveillance for the child, informs cascade testing in relatives, and shapes decisions about radiotherapy and future cancer screening.

  • Is surgery always the first step?

    Yes. Maximum safe resection by a specialist paediatric neurosurgeon is the strongest prognostic factor across all grades. For grade 1 papilloma, complete resection is often curative on its own.

  • Why is proton beam therapy preferred in children?

    Proton beam therapy delivers less radiation dose to surrounding healthy brain and body tissue. In young children this reduces the risk of long-term cognitive impairment, endocrine failure and second cancers - particularly important in Li-Fraumeni carriers.

  • What is the outlook?

    For choroid plexus papilloma with complete resection, outcomes are excellent. For carcinoma, outcomes have improved with multimodal treatment on SIOP-CPT protocols but remain more guarded, especially with metastatic disease at diagnosis. Care in a specialist centre and enrolment in international trials give the best chance.

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