Choroid Plexus Carcinoma

Overview

Choroid plexus carcinoma is a very rare type of brain tumour (a growth of abnormal cells). It starts in the choroid plexus — a small, frilly layer of tissue inside the fluid-filled spaces of the brain (called ventricles) that normally makes the cerebrospinal fluid (CSF) that cushions and protects the brain and spinal cord. In this cancer, cells from that tissue grow out of control and form a lump. Because the choroid plexus makes fluid, these tumours often block the flow of fluid, which can cause a build-up of pressure inside the head (called hydrocephalus, meaning 'water on the brain'). Choroid plexus carcinoma is the fast-growing, cancerous form of choroid plexus tumour. There is also a slower-growing, non-cancerous form called choroid plexus papilloma, which is treated differently.

No. Choroid plexus carcinoma is extremely rare. All choroid plexus tumours together make up only a small fraction of childhood brain tumours, and the cancerous form (carcinoma) is the rarest type. Because it is so uncommon, it is usually treated in specialist centres with teams who have experience of it.

Most cases are diagnosed in babies and children under 2 years old, and it affects boys and girls about equally. It can also occur in older children and, much less often, in adults. In a small number of cases it is linked to an inherited condition that raises the risk of several types of cancer, so families may be offered genetic advice.

Symptoms

Common symptoms

• Headache, often worse in the morning or when lying down
• Feeling sick or being sick, sometimes suddenly and without warning
• Unusual sleepiness, tiredness or confusion
• Changes in vision, such as blurred or double vision
• Problems with balance or walking
• Seizures (fits)

Symptoms in children

• An unusually large or quickly growing head size (in babies, the soft spot on the head may feel full or bulge)
• Downward-looking eyes, sometimes described as 'sunset eyes'
• Being very irritable, unsettled or crying more than usual
• Feeding problems, poor appetite or not putting on weight
• Delayed development or losing skills your child had before
• Weakness, clumsiness or a change in how your child uses an arm or leg
• Stiff neck or a back that arches

Symptoms in older adults

• A new, persistent headache that keeps coming back
• Memory or thinking changes, or a change in personality
• Weakness or numbness on one side of the body
• Vision changes
• Seizures
• Problems with balance or coordination

Causes

Main causes

• In most people, the cause is not known. Like many cancers, it starts when mistakes (changes) build up in the genes inside a cell, making it grow and divide out of control.
• These gene changes usually happen in the tumour cells only. They are not passed from parent to child and are not caused by anything a parent did or did not do.
• In a small number of cases, choroid plexus carcinoma is linked to a rare inherited condition that raises the risk of several cancers, such as Li-Fraumeni syndrome. A specialist can discuss testing if this is a concern.

Risk factors

• Young age — it is most common in babies and children under 2
• Having a known rare inherited condition that increases cancer risk (affects only a small minority of cases)
• There is no evidence that everyday things such as diet, screens, mobile phones, or minor head knocks cause this tumour

When to see a doctor

See a doctor urgently if:

• Any symptom from the emergency list — call your local emergency number right away
• Persistent vomiting, especially in the morning, in a baby or young child
• A baby's head size increasing quickly, or a bulging soft spot
• New eye movement changes, unusual sleepiness, or loss of skills
• A new headache that keeps returning or wakes your child from sleep

Book a routine appointment if:

• You are worried about your child's development or growth and want a check-up
• Your child has had a few unexplained episodes of vomiting or irritability
• You or your child has ongoing headaches that you would like assessed
• You have questions about family risk because a relative had a brain tumour or a known inherited cancer condition

Diagnosis

A doctor will start by asking about symptoms and doing a physical and neurological examination (checking movement, reflexes, eyes and, in a baby, head size and the soft spot). If a brain problem is suspected, they will arrange urgent brain scans. A scan can show a lump and any fluid build-up, but only a small sample of tissue (a biopsy) or tissue removed during surgery can confirm exactly what type of tumour it is.

Tests that may be done

• Brain imaging — usually an MRI scan, sometimes a CT scan first, to look at the brain and the fluid spaces
• A biopsy or tumour sample taken during surgery, examined under a microscope to confirm the tumour type
• A lumbar puncture (spinal tap) in some cases, to check whether cells have spread into the fluid around the brain and spine
• Scans of the spine, and sometimes a full body scan, to see whether the tumour has spread
• Blood and urine tests to check general health and to rule out other causes
• Genetic testing of the tumour, and sometimes a blood test, to look for inherited conditions that may affect the family

What to expect at your appointment

Tests are usually done quickly because symptoms can get worse over days or weeks. Some tests need a general anaesthetic for young children, which is routine and closely supervised. Waiting for results is often the hardest part — results may take several days, and you may be referred to a specialist children's or adult neuroscience centre. Ask your team to explain each step, and write down your questions so you don't forget them. Bringing someone with you to appointments can help you remember what was said.

Treatment

Treatment is planned by a specialist team — usually including children's cancer doctors, brain surgeons, radiologists and specialist nurses. The aim is to remove as much of the tumour as is safe, control any spread, and manage pressure and fluid build-up. Plans are tailored to the person's age, the size and position of the tumour, and whether it has spread. Because this tumour is so rare, you may be offered the chance to take part in a clinical trial, which helps doctors learn the best way to treat it.

Self-care at home

• Keep a simple symptom diary (headaches, vomiting, sleepiness, medicines given) to share with the team
• Attend all follow-up appointments and scans — they help catch problems early
• Ask the specialist nurse for written information and a single point of contact
• Keep your child's routine as normal as possible: regular meals, naps, play and school where they are well enough
• Ask about support with travel, parking, costs and time off work — hospital social workers can help
• Note that some treatments affect fertility and growth, so ask the team about long-term follow-up and what to watch for

Medical treatments

Treatment usually combines several approaches. Surgery aims to remove as much of the tumour as possible. Chemotherapy (cancer-killing medicine given by drip or as tablets) is often used, especially in young children, to shrink the tumour before or after surgery. Radiotherapy (high-energy beams targeted at the tumour) may be used in older children and adults, but is avoided where possible in very young children because it can affect a growing brain. A shunt or endoscopic procedure (a small tube or opening that lets fluid drain) may be needed to relieve pressure from fluid build-up. Steroids and other supportive medicines may be used to reduce swelling around the tumour. Your specialist team will explain which options suit your situation, including the possible benefits and side effects. Always ask your own team before making any decisions about treatment.

When is surgery considered?

Surgery is usually the first main treatment when the tumour can be reached safely. The aim is to remove as much of it as possible without harming nearby brain tissue. If the tumour is in a difficult position, or if the person is very young or unwell, the team may suggest chemotherapy first to shrink it. Sometimes a second operation is needed. Head surgery sounds frightening, but specialist teams do this regularly and plan carefully using detailed scans.

Living with this condition

Life during and after treatment usually settles into a new rhythm of appointments, rest and recovery, with good days and harder days. Children often cope better than adults expect, but they need extra reassurance, play and cuddles. Adults may need help with tiredness, memory and work. Take things one step at a time, accept offers of help, and let the specialist team know early about any new symptom rather than waiting for the next appointment.

Lifestyle tips

• Keep routines gentle and predictable — regular sleep, meals and quiet time help recovery
• Ask the team what activity is safe; gentle play and short walks are often encouraged
• Keep up vaccinations and general health checks as advised by your team
• Avoid people with infections when the immune system is low, and follow hand hygiene advice
• Return to school or work gradually, with a plan agreed with the team and, for children, the school
• Ask about physiotherapy, occupational therapy and speech therapy — they can make a real difference to day-to-day life

Diet and exercise

There is no special diet that treats this tumour, but eating well and staying as active as feels comfortable helps the body cope with treatment. Aim for regular balanced meals and snacks, and plenty of fluids. If appetite is poor or there is nausea, small frequent meals often work better than large ones, and a dietitian can give practical ideas. Gentle movement — short walks, stretching, playing — is usually helpful when the person feels up to it. Ask your team before starting anything strenuous, especially after surgery.

Mental health and emotional wellbeing

A rare brain tumour diagnosis is frightening for the whole family. It is normal to feel shock, grief, anger, guilt, or to feel numb. Children may show their feelings through behaviour, clinginess or regression, and siblings may feel left out or worried. These feelings do not mean anyone is coping badly — they are part of a very hard experience. Talking helps: to each other, to the specialist nurse, to a psychologist attached to the cancer team, or to a support group. If you or your child feels persistently low, hopeless or unable to cope, please reach out to your healthcare team or a crisis support service straight away. You do not have to manage this alone.

Prevention

There is no known way to prevent choroid plexus carcinoma. It is not caused by anything parents did or did not do, and it is not linked to lifestyle choices such as diet, exercise or screen time. Because it is so rare, there is no routine screening test for it either. If a rare inherited cancer condition runs in your family, genetic counselling can help you understand your family's risk and what monitoring may be appropriate.

Screening programmes

There is no screening programme for choroid plexus carcinoma. In families with a known inherited cancer syndrome, doctors may recommend tailored check-ups — a genetics specialist can advise.

Complications

If left untreated

• Rising pressure inside the head from a build-up of fluid (hydrocephalus), which can cause severe headaches, vomiting and drowsiness
• The tumour growing and pressing on nearby brain areas, affecting movement, vision, speech or behaviour
• Spread of tumour cells through the fluid around the brain and spinal cord
• In young children, delayed development or loss of skills if pressure is not relieved
• In serious cases, dangerous pressure on the brain — this is a medical emergency

Long-term outlook

Choroid plexus carcinoma is a serious tumour, and the honest picture is that it grows quickly and needs prompt, intensive treatment. But outlook has improved as surgery, chemotherapy and specialist care have got better, and many people, especially children, do well with combined treatment, particularly when the tumour can be largely removed. Because the tumour is rare, outcomes vary a lot between individuals, and every case is different. Your specialist team can talk you through what is known about your or your child's specific situation. Whatever the stage, there is always support available — medical, practical and emotional — and reasons for hope.

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