Choroid plexus carcinoma is a rare, fast-growing brain cancer that starts in the choroid plexus, the soft tissue inside the brain’s ventricles that makes cerebrospinal fluid (CSF). In this cancer, the cells of the choroid plexus grow in a very abnormal way, form a mass (tumor), and often block the normal flow of CSF, which can cause a build-up of pressure in the brain called hydrocephalus. Doctors call it a World Health Organization (WHO) grade 3 choroid plexus tumor, which means it is a high-grade, aggressive cancer. It happens mostly in babies and young children, but it can also occur in older children and adults, although that is much less common.
Choroid plexus carcinoma (CPC) is a rare, fast-growing brain cancer that starts in the choroid plexus, the tissue inside the brain that makes cerebrospinal fluid (CSF). It happens mostly in babies and young children, but can also affect older people. The tumor can block the flow of CSF and raise pressure inside the skull, which causes headache, vomiting, and changes in behavior or movement. Treatment usually needs surgery, chemotherapy, and sometimes radiotherapy, given in a specialized children’s cancer center by an expert team. [1]
This tumor can spread along the flow of cerebrospinal fluid to other parts of the brain and spinal cord, but it usually does not spread outside the central nervous system. Because it grows quickly and can spread, it needs fast and strong treatment, usually with surgery and other therapies.
Other names
Doctors and researchers may use several different names that all point to the same disease. Knowing these names can help you understand test reports or research papers.
Grade 3 choroid plexus tumor
WHO grade III choroid plexus carcinoma
Choroid plexus cancer
Malignant choroid plexus tumor
High-grade choroid plexus tumor
All these names mean that the tumor is cancerous (malignant) and is the most serious type in the choroid plexus tumor family.
Types
Doctors usually talk about choroid plexus tumors as a group and then separate them by grade. Within this group, choroid plexus carcinoma is one specific type.
Main types of choroid plexus tumors are:
Choroid plexus papilloma (CPP) – WHO grade I, a low-grade, slow-growing, non-cancerous tumor.
Atypical choroid plexus papilloma (aCPP) – WHO grade II, still low-grade but more active and more likely to come back than CPP.
Choroid plexus carcinoma (CPC) – WHO grade III, the high-grade cancerous form that grows quickly and can invade nearby brain tissue and spread in CSF.
Choroid plexus carcinomas can also be described by location: for example, tumors in the lateral ventricle (more common in children), tumors in the fourth ventricle (more often in adults), or rarely tumors in other or extraventricular sites.
Causes and risk factors
For most people with choroid plexus carcinoma, the exact cause is not known. Cancer happens when genes inside cells change (mutate) and make the cells grow out of control. Below are important known and suspected causes and risk factors.
Unknown (sporadic) genetic changes in tumor cells
In many patients, doctors cannot find any clear outside cause. The tumor seems to come from random harmful changes in the DNA of choroid plexus cells. These changes make the cells divide too much, live too long, and form a mass. This is called a “sporadic” cancer, meaning it arises by chance without a clear trigger.TP53 gene mutation
The TP53 gene helps control cell growth and repair damaged DNA. In about half of choroid plexus carcinomas, tumor cells show TP53 mutations. When TP53 is damaged, abnormal cells are not repaired or destroyed, so they keep growing and can turn into cancer.Li-Fraumeni syndrome (inherited TP53 mutation)
Some children and adults are born with a TP53 mutation in all their cells, a condition called Li-Fraumeni syndrome. These people have a much higher risk of several cancers, including brain tumors such as choroid plexus carcinoma. Not all people with Li-Fraumeni develop this tumor, but their risk is clearly higher than normal.Other inherited cancer-predisposition syndromes
Choroid plexus carcinomas have also been reported in children with other hereditary syndromes that affect DNA repair or cell growth, such as some mismatch-repair defects and other rare cancer-predisposition conditions. In these syndromes, the overall ability of the body to correct DNA damage is reduced, which can increase cancer risk.Family history of early brain tumors
When several close family members have had childhood brain tumors or early-onset cancers, it may show an inherited tendency to develop cancers, including choroid plexus tumors. In such families, genetic counselling and testing may be recommended.Very young age (especially under 2 years)
Choroid plexus carcinoma is most common in very young children, especially under 2 years old. Age itself is not a “cause,” but it shows when the risk is highest, possibly because quickly growing brain tissue is more sensitive to genetic problems.Rapid brain growth and active choroid plexus in infancy
In babies, the choroid plexus is very active and makes a lot of cerebrospinal fluid. This high activity means the cells are dividing and working more, which may make it easier for DNA errors to happen and accumulate, which could lead to cancer in rare cases.Previous radiation to the brain
In general brain-tumor research, exposure to ionizing radiation to the head, especially in childhood, is linked to a higher risk of later brain tumors. While this is not specific only to choroid plexus carcinoma, it is considered a possible risk factor for many brain cancers.Environmental DNA-damaging factors (possible)
Strong radiation or certain toxic chemicals can damage DNA and may contribute to cancer, but for choroid plexus carcinoma there is no consistent proof of any specific environmental chemical cause. These factors are considered possible, but not clearly proven.Defects in DNA repair pathways
Some rare inherited or acquired problems in genes that repair DNA damage (for example mismatch-repair genes) can lead to a build-up of mutations in many cells, including brain cells. This general problem can raise the risk of various cancers, including rare tumors such as choroid plexus carcinoma.Abnormal cell-cycle control in choroid plexus epithelium
Tumor studies show high cell-division rates (high Ki-67 index) in choroid plexus carcinoma. This means the internal “stop and go” signals that control cell division are disturbed, so cells keep dividing when they should stop, which helps the tumor grow.Imbalance in growth signals and death signals
Normal cells receive signals that tell them when to grow and when to die. In choroid plexus carcinoma, growth-promoting signals are stronger and death-promoting signals are weaker. This imbalance makes the tissue overgrow and form a tumor.Abnormal blood supply and micro-environment in the ventricles
The choroid plexus has a rich blood supply and sits in a fluid-filled space. Changes in local blood vessels or surrounding fluid factors may support survival of abnormal cells and make it easier for them to expand.Spread from a pre-existing choroid plexus papilloma (rare)
Very rarely, a previously benign or atypical choroid plexus papilloma may show more aggressive changes and behave like a carcinoma. This “malignant transformation” is uncommon but has been reported in the broader choroid plexus tumor literature.Sex-related factors in choroid plexus tumors
Some studies suggest that choroid plexus tumors as a group may appear slightly more often in females, though the reason is not clear. Hormonal or genetic sex differences may possibly influence tumor risk, but this is still being studied.Hydrocephalus-related pressure and tissue stress (secondary effect)
Long-standing increased pressure inside the ventricles can stretch and stress the choroid plexus. While hydrocephalus is usually a result of the tumor, ongoing tissue stress might contribute to further local damage and abnormal growth in some cases.Immune system surveillance problems
The brain is partly protected from the immune system by the blood-brain barrier. If immune surveillance in the central nervous system is not strong enough, abnormal choroid plexus cells may survive instead of being removed by the body’s defenses, which could allow tumor formation.Complex interaction of many small genetic variants
Instead of one big mutation, many small inherited genetic differences may together slightly increase the risk of rare tumors. On their own, each change is weak, but together they can make the tissue a bit more likely to develop cancer if more DNA damage occurs.Possible role of somatic mosaicism
Sometimes a harmful mutation happens early in development in just some cells, including those that become the choroid plexus. This is called somatic mosaicism. These cells then carry a higher risk of turning into a tumor later, even when the rest of the body is normal.Still-unknown biological factors
Even with modern testing, many children with choroid plexus carcinoma have no known risk factor or inherited syndrome. This tells us that there are still unknown genetic or biological causes that researchers have not yet discovered.
Symptoms and signs
Symptoms usually come from increased pressure inside the skull and blockage of cerebrospinal fluid, rather than from the tumor itself. Symptoms can be different in babies, children, and adults, but many are related to hydrocephalus and pressure on brain tissue.
Headache, often worse in the morning
Many patients, especially older children, have strong headaches that are more severe when they wake up. Pressure in the skull is often higher after lying down all night, so the morning is the worst time. Headaches may improve a little during the day but then come back.Nausea and vomiting
Increased pressure inside the skull can irritate the brain areas that control nausea and vomiting. Children may vomit in the morning without feeling sick to their stomach first, or they may have repeated vomiting with no clear stomach infection.Lethargy and excessive sleepiness
A child may seem unusually sleepy, tired, or hard to wake. They may lose interest in playing and may sleep much more than normal. This often shows that the brain is under pressure and not working normally.Irritability and behavior changes
Babies and young children may become very fussy, cry more than usual, or be difficult to comfort. Older children may have mood swings, become more withdrawn, or show changes in school performance or personality.Enlarged head size in infants
In babies whose skull bones are not yet fused, increased pressure can make the head grow faster than normal. Parents or doctors may notice a rapidly increasing head circumference or a bulging soft spot (fontanelle) on the top of the head.Bulging or tense fontanelle (soft spot)
The soft area on top of a baby’s head may look swollen or feel tight and firm instead of soft and slightly sunken. This is a warning sign of increased pressure in the brain.Problems with feeding or poor weight gain
Babies may refuse feeds, vomit after feeds, or not gain weight as expected. This can happen because they feel nauseated or tired, or because pressure in the brain affects their ability to suck and swallow properly.Balance and walking problems (ataxia)
Older infants and children may stumble, fall more often, or have trouble sitting or walking steadily. The tumor and the raised pressure can affect parts of the brain that control coordination and posture.Abnormal eye movements or double vision
Some children develop rapid, jerky eye movements, eyes that do not move together, or double vision. This happens when the tumor or pressure affects the nerves that move the eyes or parts of the brain that control eye movements.Blurred or reduced vision
Swelling of the optic nerve (papilledema) from increased intracranial pressure can cause blurred vision, decreased visual field, or trouble seeing clearly. If not treated, this can sometimes lead to permanent vision damage.Seizures (fits)
Some patients have seizures, which may look like staring spells, shaking of part or all of the body, or sudden loss of awareness. Seizures happen when abnormal electrical activity spreads through brain tissue irritated by the tumor or high pressure.Weakness or numbness in arms or legs
If the tumor presses on brain areas that control movement or feeling, a child may have weakness, clumsiness, or numbness on one side of the body or both. This can make walking, using the hands, or playing more difficult.Developmental delay or regression
Babies and toddlers may not reach milestones like sitting, walking, or talking at the expected age. In some cases, a child who had already learned a skill may lose it, such as stopping walking or talking. This may reflect ongoing brain damage from pressure or tumor growth.Endocrine or growth problems (rare)
If the tumor or pressure affects brain regions near hormone-controlling centers, there may be changes in growth, puberty, or other hormone-related functions. These are less common but can occur in some brain tumor patients.Signs of raised intracranial pressure on exam
Doctors may see specific signs such as swelling of the optic disc on eye exam, high blood pressure with slow heart rate, or reduced level of consciousness in severe cases. These are serious warning signs that need urgent care.
Diagnostic tests
Diagnosing choroid plexus carcinoma usually involves several steps: a careful history, physical and neurological exam, imaging tests, and tests of tissue and fluid. The goal is to confirm that a tumor is present, understand its type and grade, and see whether it has spread in the brain or spinal cord.
Physical exam tests
Detailed medical history and symptom review
The doctor asks about headache pattern, vomiting, changes in behavior, development, seizures, and family history of cancer. This helps to understand how long the problem has been present and how fast it is getting worse, and it guides which tests are needed next.General physical examination
The doctor looks at overall health, weight, growth, skin, and other body systems. This broad check can show if there are signs of other illnesses, inherited syndromes, or treatment-related problems that could affect care.Neurological examination
The neurological exam checks strength, reflexes, sensation, coordination, balance, eye movements, and mental status. Any weakness, abnormal reflexes, or coordination problems may point to pressure on specific brain areas and support the need for urgent brain imaging.Head circumference measurement in infants
In babies, the doctor measures the head and compares it to age-based charts. A head that is growing too fast or is much larger than expected can be a sign of hydrocephalus from a tumor blocking cerebrospinal fluid flow.Developmental and cognitive assessment
The clinician checks age-appropriate skills such as sitting, walking, talking, and problem solving. Loss of skills or delayed milestones may suggest long-standing brain pressure or injury from a tumor and can help judge the severity of the disease.
Manual (bedside) tests
Eye and vision examination (including fundoscopy)
Using a light and a special lens, the doctor looks at the back of the eye to see the optic nerve. Swelling of this nerve (papilledema) is a classic sign of increased pressure in the skull, which often occurs when a choroid plexus tumor blocks fluid flow.Motor strength testing of arms and legs
The doctor asks the child to push and pull against resistance and observes how they move. Weakness on one side or in certain muscle groups may show which part of the brain or spinal cord is affected by the tumor.Coordination and balance tests (gait, finger-to-nose)
The child may be asked to walk normally, walk on heels or toes, or touch finger to nose. Unsteady walking, wide-based gait, or difficulty with precise movements can point to cerebellar or brainstem involvement, which is common when tumors affect CSF pathways.Sensory testing (touch, pain, temperature)
Gently testing how the child feels light touch, pinprick, or temperature changes can show if any part of the body has lost normal sensation. This helps localize potential compression or damage within the nervous system.Bedside mental status and behavior check
Simple questions and tasks are used to see how well the child is thinking, speaking, and understanding. Changes in alertness, memory, or behavior can support the suspicion of a serious brain problem and the need for imaging and specialist care.
Lab and pathological tests
Basic blood tests (complete blood count and chemistry)
Blood tests check overall health, look for anemia or infection, and measure kidney and liver function. These tests do not diagnose the tumor directly but are important for planning imaging with contrast and later treatments like surgery and chemotherapy.Cerebrospinal fluid (CSF) analysis and cytology
In some cases, doctors take a sample of cerebrospinal fluid, usually by lumbar puncture, to look for tumor cells that have spread through the fluid. Under a microscope, pathologists can sometimes see cancer cells from choroid plexus carcinoma in the CSF. This shows that the tumor has seeded along the CSF pathways.Tumor biopsy and histopathology
The most important test is examining the tumor tissue itself. During surgery, the tumor (or part of it) is removed and studied under a microscope. Pathologists look at how the cells are arranged, how abnormal they look, and whether they invade surrounding tissue to confirm the diagnosis of choroid plexus carcinoma.Immunohistochemistry on tumor tissue
Special stains are applied to the tumor sample to detect certain proteins, such as cytokeratins, transthyretin, and others, that are typical for choroid plexus epithelium. These stains help distinguish choroid plexus carcinoma from other brain tumors that can look similar.Genetic and molecular testing (e.g., TP53)
Molecular tests can check for TP53 and other gene changes in the tumor or in blood. Finding a TP53 mutation in the tumor supports the diagnosis and may also suggest that the patient should be evaluated for Li-Fraumeni syndrome or other inherited conditions, which affects long-term follow-up.
Electrodiagnostic tests
Electroencephalogram (EEG)
If seizures are present or suspected, an EEG records electrical activity in the brain through small electrodes on the scalp. Abnormal patterns on EEG support the presence of a brain disorder, though they do not prove the type of tumor. They help manage seizures and monitor response to treatment.Evoked potential studies (visual and brainstem auditory)
These tests measure the brain’s response to visual or sound stimuli. Delayed or abnormal signals can show that pathways from the eyes or ears through the brain are affected, giving more information about how far the tumor or pressure has disturbed brain function.
Imaging tests
Cranial ultrasound in infants
In very young babies with open fontanelles, doctors can use an ultrasound probe on the soft spot to see inside the brain. This simple bedside test can show enlarged ventricles and sometimes a mass, and it is often used as an early screening tool before more detailed scans.CT scan of the brain with contrast
A computed tomography (CT) scan uses X-rays and a computer to produce cross-section pictures of the brain. Choroid plexus tumors often appear as lobulated, vividly enhancing masses within the ventricles. CT is quick and useful in emergencies to detect hydrocephalus and guide urgent care.MRI of the brain and spine with contrast
Magnetic resonance imaging (MRI) is the main imaging test to evaluate choroid plexus carcinoma. It shows detailed views of the tumor, its relationship to brain structures, and the presence of spread along the spinal fluid pathways. Contrast dye makes the tumor stand out more clearly. MRI of the spine may be done to check for metastases along the spinal cord.
Non-Pharmacological Treatments (Therapies and Others)
1. Multidisciplinary tumor board care
A multidisciplinary tumor board is a meeting where many specialists (neurosurgeon, pediatric oncologist, radiation oncologist, radiologist, pathologist, rehab doctor, nurse, psychologist) discuss each child’s case together. The purpose is to design one clear plan that fits the child’s age, tumor size, genetic changes (like TP53), and family situation. The mechanism is simple: decision-making is shared, so important details are less likely to be missed, and treatment steps (surgery, chemo, radiotherapy, rehab) are better coordinated. [2]
2. Detailed family education and counseling
Education sessions give parents and older children clear information about the tumor, tests, treatment options, and possible side effects. The purpose is to reduce fear, help the family ask good questions, and support informed consent. The mechanism is communication: doctors and nurses use simple words, pictures, and written plans so the family understands what will happen next, how to give medicines at home, and when to return to hospital if there is a problem.
3. Physical therapy (physiotherapy)
Physical therapy uses exercises, stretching, and safe movement training to keep muscles strong and joints flexible. The purpose is to prevent stiffness, weakness, and loss of balance that can happen after brain surgery or chemotherapy. The mechanism is repeated practice: simple tasks like standing, walking, and climbing steps are broken into small steps. Over time, the brain learns new movement paths and the body becomes stronger and more stable.
4. Occupational therapy
Occupational therapy focuses on daily life skills, such as dressing, feeding, writing, using a phone, or playing. The purpose is to help the child stay as independent as possible at home and at school. The mechanism is adaptation: the therapist teaches new ways to do tasks, suggests special tools (bigger handles, special spoons), and helps the family change the home to make it safer and easier to move around.
5. Speech and language therapy
Speech therapy helps children who have trouble speaking, understanding words, swallowing, or communicating after brain surgery or treatment. The purpose is to improve safety (for example, avoiding choking) and to support learning and social life. The mechanism is targeted exercises for muscles of the mouth and face, plus language games and communication tools (picture boards, tablets) to support clear, safe swallowing and better speech.
6. Neuropsychological assessment and cognitive rehabilitation
A neuropsychologist tests memory, attention, learning, and behavior using child-friendly tasks. The purpose is to detect early thinking problems caused by the tumor, surgery, chemotherapy, or radiotherapy. The mechanism is measurement and training: the team creates a rehabilitation plan with memory games, attention training, and school strategies so the child can keep learning and stay engaged in class. [3]
7. School support and individualized education plans
School support means working closely with teachers, school counselors, and special education staff. The purpose is to help the child stay in school when possible or return safely after treatment. The mechanism is accommodation: the team may recommend shorter school days, extra rest breaks, extra time for exams, or a classroom assistant. Clear communication between hospital and school prevents misunderstandings and bullying.
8. Nutritional counseling
A dietitian helps the family plan meals that give enough calories, protein, vitamins, and fluids, even when the child feels sick, tired, or has taste changes. The purpose is to keep weight stable and support healing and immune function. The mechanism is adjusting food texture, flavor, and timing, and suggesting snacks, smoothies, or medical nutrition drinks when usual eating is difficult.
9. Seizure safety and home environment modification
Some children with CPC have seizures. Seizure safety means teaching the family what to do during a seizure and how to make the home safer. The purpose is to prevent injury. The mechanism includes avoiding high, sharp edges, not leaving a child alone in deep water, and using helmets or bed rails when recommended. The team also teaches when to call emergency services.
10. Non-drug pain and nausea management
Non-drug methods include cool cloths, relaxation breathing, distraction with music or games, gentle massage, and comfortable positioning in bed. The purpose is to reduce pain and nausea without adding more medicines when possible. The mechanism is mind-body: calming the nervous system, shifting attention away from discomfort, and reducing muscle tension, which can lower the feeling of pain.
11. Psychological therapy (child and family)
Psychologists or counselors offer talk therapy, play therapy, or art therapy. The purpose is to help children and parents express fear, sadness, or anger, and to build coping skills. The mechanism is emotional support: naming feelings, learning simple breathing or relaxation exercises, and challenging frightening thoughts so they feel less strong and overwhelming.
12. Social work support
Social workers help families with practical needs such as transport, financial help, paperwork, and community resources. The purpose is to reduce stress from money or travel problems so the family can focus on the child’s care. The mechanism is problem-solving and connection: linking the family with charities, government programs, or housing help when necessary.
13. Spiritual or cultural support (if the family wishes)
Some families find comfort in spiritual or cultural practices. Hospital chaplains or community leaders can visit if the family requests this. The purpose is to support meaning, hope, and strength. The mechanism is listening, rituals, and presence, always guided by what the family wants, never forced.
14. Palliative care team involvement (early, not only at the end)
Palliative care teams focus on comfort, symptom control, and quality of life, starting early in the illness. The purpose is to control pain, nausea, sleep problems, and emotional distress, even while the child is still receiving active cancer treatment. The mechanism is careful assessment, regular check-ins, and coordination with the oncology team to adjust support as needs change. [4]
15. Sleep hygiene and daily routine planning
Good sleep habits include regular bedtimes, quiet, dark rooms, and reducing screens before sleep. The purpose is to improve energy, mood, and attention. The mechanism is stabilizing the body clock: a predictable routine helps the brain settle and allows better healing and daytime functioning.
16. Early intervention programs for infants and toddlers
For very young children, early intervention services offer home-based play therapy, physiotherapy, and speech support. The purpose is to support brain development during a critical time. The mechanism is frequent, gentle stimulation and parent coaching, helping the child reach milestones as well as possible.
17. Rehabilitation after surgery and intensive care
After a big brain surgery, children may be weak, confused, or have new movement or speech problems. Inpatient rehabilitation combines physical, occupational, and speech therapy. The purpose is to regain function as quickly and safely as possible. The mechanism is repetitive, goal-based exercises with close medical supervision.
18. Radiotherapy planning and immobilization support (as a process)
Radiotherapy is a key non-drug treatment that uses focused radiation beams to kill remaining tumor cells after surgery or chemotherapy. The purpose is to reduce the chance of tumor regrowth. The mechanism is DNA damage inside cancer cells, which stops them dividing. Children may need a custom mask or mold to keep the head still, and support to stay calm during each short session. [5]
19. Regular imaging and follow-up visits
Follow-up MRI scans and clinic visits help doctors detect early signs of tumor recurrence or treatment side effects. The purpose is early action if changes appear. The mechanism is monitoring: comparing new scans to old ones and checking growth, school performance, and hormone function.
20. Long-term survivorship programs
Survivorship clinics follow children for years after treatment ends. The purpose is to watch for late effects on thinking, hearing, hormones, heart, and other organs. The mechanism is scheduled tests (hearing checks, hormone tests, cognitive assessments) and timely referrals so that any new problem is treated early. [6]
Drug Treatments
⚠️ Very important: The drugs below are examples used by specialist teams in hospitals. Doses and schedules are never self-decided. They are calculated by pediatric oncology experts using body surface area, age, kidney and liver function, and many other factors. This information is for general education only, not for self-treatment.
Most chemotherapy drugs used in choroid plexus carcinoma are approved by the U.S. FDA for other cancers and are used “off-label” in expert pediatric brain tumor protocols. [7]
1. Cisplatin
Cisplatin is a platinum-based chemotherapy drug. It forms strong cross-links in DNA, which stops cancer cells from copying their genetic material and causes them to die. The purpose in CPC is to kill fast-dividing tumor cells after or around surgery. Dose and timing are decided by oncologists, usually in cycles with rest periods. Common side effects include nausea, kidney damage, hearing loss, and low blood counts, so strong monitoring and hydration are essential. [8]
2. Carboplatin
Carboplatin is another platinum-based drug with similar DNA-damaging effects but a different side-effect profile. It is often used in protocols such as ICE (ifosfamide, carboplatin, etoposide) for choroid plexus carcinoma. The purpose is to work together with other agents to improve tumor control. Dosage is based on kidney function and body size and is planned in cycles. Side effects include low blood counts, nausea, and less kidney and hearing damage than cisplatin, but still needs close monitoring. [9]
3. Ifosfamide
Ifosfamide is an alkylating chemotherapy agent. It attaches alkyl groups to DNA, causing breaks and preventing cancer cell division. In CPC, it is used in combination regimens like ICE after surgery. The purpose is to deepen tumor cell killing. Dose and schedule are carefully controlled in hospital. Side effects include low blood counts, kidney and bladder irritation, and confusion; protective medicines and extra fluids are given to reduce bladder damage.
4. Cyclophosphamide
Cyclophosphamide is another alkylating agent that damages DNA inside dividing cells. It is part of several pediatric brain tumor protocols. The purpose in CPC is to help clear microscopic tumor cells that surgery cannot remove. Dosage is based on body surface area and may be given intravenously or orally in cycles. Side effects include low white cells, hair loss, nausea, and, rarely, heart or bladder problems, so careful blood and urine tests are required. [10]
5. Etoposide
Etoposide is a topoisomerase II inhibitor. It blocks an enzyme that helps unwind DNA, leading to DNA damage and cancer cell death. In CPC, it is used with platinum drugs or ifosfamide. The purpose is to attack tumor cells in a different way than alkylators, improving the chance of response. It is given by vein or as capsules, in cycles. Side effects include low blood counts, hair loss, nausea, and, rarely, secondary leukemias after long-term use. [11]
6. Vincristine
Vincristine is a vinca alkaloid that disrupts microtubules, the “skeleton” inside dividing cells. This stops cells from completing mitosis. In CPC, it is used in pediatric brain tumor regimens along with other drugs. The purpose is to add another attack point on the tumor. Vincristine is given intravenously once a week or as planned in protocols. Side effects include nerve damage (tingling, weakness), constipation, and jaw pain; it must never be given into the spine. [12]
7. High-dose methotrexate
Methotrexate blocks folate pathways needed to build DNA. At high doses, it can cross into the cerebrospinal fluid and reach tumor cells near the ventricles. In CPC, high-dose methotrexate is sometimes used in intensive regimens like “Head Start” protocols. The purpose is to deliver strong chemotherapy to the brain without immediate radiotherapy in very young children. It requires hospital admission, rescue with folinic acid, and strict monitoring for kidney and liver toxicity. [13]
8. Temozolomide
Temozolomide is an oral alkylating agent used mainly for gliomas but also tried in some CPC cases, especially at relapse. It crosses the blood–brain barrier and damages tumor DNA. The purpose is to provide a convenient, oral option that can be combined with radiotherapy or other drugs. Dose schedules vary and are decided by specialists. Side effects include low blood counts, fatigue, nausea, and risk of infection.
9. Topotecan
Topotecan is a topoisomerase I inhibitor. It interferes with the DNA repair process, causing cancer cell death. It has been used in intraventricular or systemic chemotherapy for recurrent CPC in some studies. The purpose is to target residual or relapsed tumor cells when standard options are limited. Side effects include low blood counts, diarrhea, and fatigue, so careful hospital monitoring is needed. [14]
10. Thiotepa
Thiotepa is a strong alkylating agent used especially in high-dose chemotherapy with autologous stem cell rescue. The purpose is to intensively treat remaining tumor cells before stem cells are given back. It crosses into the CNS and can reach tumor cells near CSF spaces. Side effects include profound low blood counts, skin irritation from drug excreted in sweat, and liver toxicity, so it is only used in highly experienced centers.
11. Lomustine (CCNU)
Lomustine is an oral nitrosourea that crosses the blood–brain barrier. It alkylates DNA in tumor cells. In CPC, it may be used in salvage or maintenance regimens. The purpose is long-lasting, brain-penetrating chemotherapy that can be taken by mouth. Side effects include delayed low blood counts, nausea, and risk of lung or liver toxicity, so blood tests and follow-up imaging are necessary.
12. Procarbazine
Procarbazine is another oral alkylating drug often used with lomustine and vincristine in brain tumor combinations. The purpose in CPC is to provide a multi-drug oral regimen targeting tumor DNA. Side effects include low blood counts, nausea, and sensitivity to certain foods and medicines, so families must follow strict safety advice from the oncology team.
13. Dexamethasone
Dexamethasone is a corticosteroid, not a chemotherapy drug, but is crucial in CPC care. It reduces swelling around the tumor and lowers intracranial pressure. The purpose is to quickly improve headache, vomiting, and neurological symptoms. Dose and timing are short-term and carefully tapered. Side effects include mood changes, high blood sugar, infection risk, and stomach irritation, so it is used only when needed and then reduced. [15]
14. Mannitol and hypertonic saline
These are medicines used in emergency situations to reduce dangerous brain swelling. They work by pulling fluid out of brain tissue into the bloodstream. The purpose is short-term control of life-threatening raised pressure before or after surgery. Doses are given in intensive care with very close monitoring of blood pressure, electrolytes, and kidney function.
15. Levetiracetam
Levetiracetam is a common anti-seizure drug. It acts on brain cell channels to decrease abnormal electrical activity. In CPC, it is used to prevent or treat seizures caused by the tumor or surgery. Dose is based on weight and adjusted by blood levels and side effects. Common problems include sleepiness, irritability, or mood changes, so behavior is watched carefully.
16. Ondansetron and similar anti-nausea drugs
Ondansetron blocks serotonin receptors in the brain and gut to reduce chemotherapy-induced nausea and vomiting. The purpose is to help children tolerate chemotherapy and stay hydrated. It is given before and sometimes after chemotherapy. Side effects are usually mild, like headache or constipation, but heart rhythm is monitored if high doses are used. [16]
17. Proton pump inhibitors (e.g., omeprazole)
These drugs lower stomach acid. The purpose in CPC treatment is to protect the stomach when steroids or some chemotherapy drugs are used, reducing the risk of ulcers and heartburn. Doses depend on age and weight. Side effects can include diarrhea, headache, and, with long-term use, small changes in mineral absorption; doctors try to use the lowest effective dose for the shortest time.
18. Broad-spectrum antibiotics (various classes)
Children receiving intensive chemotherapy often need antibiotics when they have fever and very low white cells. The purpose is to treat or prevent serious bacterial infections quickly. The specific antibiotic and dosage are chosen by hospital guidelines and culture results. Side effects depend on the drug but can include allergic reactions, diarrhea, or kidney or liver strain, so lab tests and clinical monitoring are routine.
19. Antifungal medications (e.g., fluconazole)
Antifungal drugs may be used to prevent or treat fungal infections in very immunocompromised patients. The purpose is to protect children whose immune systems are weakened by chemotherapy. Doses and duration depend on risk level and lab tests. Side effects include liver enzyme changes and drug interactions, so blood is checked often.
20. Pneumocystis pneumonia prophylaxis (e.g., trimethoprim-sulfamethoxazole)
Low-dose antibiotic prophylaxis helps prevent a serious lung infection called Pneumocystis pneumonia in children receiving high-dose steroids or chemotherapy. The purpose is infection prevention rather than treatment. The schedule is usually a few days each week, tailored to the child. Side effects can include rash, low blood counts, or allergy, so the team monitors carefully and changes drugs if needed.
Dietary Molecular Supplements
⚠️ Supplements do not cure CPC. They may support general health but must only be used if the oncology team agrees, to avoid dangerous interactions.
1. Vitamin D
Vitamin D helps bone health, immune function, and muscle strength. Chemotherapy, steroids, and reduced sunlight can lower vitamin D levels. The purpose of supplementation is to keep blood levels in a healthy range to protect bones and general health. Dose is based on blood tests and age; doctors choose a safe daily or weekly amount. The mechanism is regulation of calcium and phosphorus balance and support of immune cell activity.
2. Calcium
Calcium supports bones, teeth, and muscle function. Children on steroids or with low mobility are at risk of weak bones. Supplementation is used when diet and vitamin D are not enough. Dose is individualized and may be split during the day. Calcium works by providing the mineral building blocks that bones need; too much can cause constipation or kidney stones, so medical advice is essential.
3. Omega-3 fatty acids (fish oil or plant sources)
Omega-3 fats may help with inflammation, heart health, and brain function. In CPC care, they are sometimes considered to support general health and appetite. Dose is chosen by the doctor or dietitian to avoid blood-thinning effects at high levels. The mechanism involves changing cell membranes and signaling molecules that control inflammation.
4. Probiotics (selected strains)
Probiotics are “good” bacteria that may help keep the gut microbiome balanced, especially during and after antibiotics. The purpose is to reduce diarrhea and support digestion. Only specific, studied products and doses should be used in children with low immunity, and some children should not receive live bacteria at all, so oncologist approval is critical.
5. Zinc
Zinc is a trace mineral important for wound healing and immune function. Poor appetite and chemotherapy can reduce intake. Supplementation can be used when blood levels are low. Dose is based on age and lab results; too much zinc can interfere with copper balance. Zinc helps enzymes and immune cells work correctly.
6. Selenium
Selenium supports antioxidant enzymes that protect cells from oxidative stress. Careful, low-dose supplementation may be used when dietary intake is poor. The mechanism is support of glutathione peroxidase and other enzymes that clear harmful reactive oxygen species. High doses are toxic, so only doctor-guided supplementation is safe.
7. B-complex vitamins (including folate and B12)
B vitamins help energy production, nerve function, and blood cell formation. In CPC treatment, diet changes and some drugs can affect these vitamins. A balanced B-complex supplement may be used when deficiency is proven or strongly suspected. Dose is chosen according to age and lab tests. The mechanism is support of many metabolic pathways in cells.
8. Iron (only if iron-deficiency is present)
Iron is essential for hemoglobin and oxygen transport. Many CPC patients are anemic from chemotherapy, but iron supplements are only useful if iron deficiency is present; otherwise, they do not help and may cause side effects. Doctors use blood tests to decide about dose and timing. Iron works by providing raw material for new red blood cells.
9. L-glutamine
L-glutamine is an amino acid that can support gut and muscle cells. Some centers use it to try to reduce mouth sores or gut side effects from chemotherapy, but evidence is mixed. Doses and duration are chosen by specialists. The mechanism is providing fuel for rapidly dividing cells and supporting the gut barrier.
10. Medical nutrition drinks (balanced formulas)
These are ready-made liquid supplements with calories, protein, vitamins, and minerals. The purpose is to help when a child cannot eat enough solid food. The dose is measured in milliliters or bottles per day, decided by a dietitian. The mechanism is simple: concentrated nutrition supports energy, tissue repair, and immune function while the child is recovering.
Immunity-Booster / Regenerative / Stem Cell–Related Drugs
These therapies support the blood and immune system. They are not general “immune boosters” for home use; they are hospital medicines with strong effects and risks.
1. Filgrastim (G-CSF)
Filgrastim is a lab-made form of granulocyte colony-stimulating factor, a natural body signal. It tells the bone marrow to make more neutrophils (a type of white blood cell). The purpose is to shorten the time of low white cells after chemotherapy and reduce infection risk. Dose is based on weight and given as injections. Side effects include bone pain and occasional spleen enlargement.
2. Pegfilgrastim
Pegfilgrastim is a long-acting form of G-CSF. It works in a similar way but lasts longer, so it is given less often. The purpose is convenience and steady support of neutrophil recovery after certain chemotherapy cycles. Its mechanism is prolonged stimulation of neutrophil production. Dosing is carefully timed after chemotherapy; side effects are similar to filgrastim.
3. Epoetin alfa (erythropoietin)
Epoetin alfa is a synthetic form of a hormone that tells the bone marrow to make more red blood cells. In some long, intensive treatments with severe anemia, it may be used to reduce transfusion needs. Dose depends on weight and hemoglobin level. The mechanism is stimulation of red cell precursors; side effects include high blood pressure or clot risk, so it is used cautiously.
4. Thrombopoietin receptor agonists (e.g., eltrombopag)
These medicines stimulate platelet production in the bone marrow by activating the thrombopoietin receptor. In selected cases with low platelets, they may help reduce bleeding risk. Dose is based on weight and liver function. The mechanism is increased platelet formation; side effects can include liver enzyme changes or clot risk, so close monitoring is needed.
5. Intravenous immunoglobulin (IVIG)
IVIG is a purified antibody product made from pooled donor plasma. It can support immunity in some children with low antibody levels or certain immune complications. The purpose is to provide ready-made antibodies for temporary protection. It is given by infusion over several hours. Side effects can include headache, fever, or, rarely, kidney issues or clots; dose and interval are carefully planned.
6. Autologous hematopoietic stem cell transplant (HSCT)
In some very high-risk or relapsed CPC cases, high-dose chemotherapy followed by autologous HSCT is used. Doctors collect the child’s own blood stem cells, store them, give very strong chemotherapy to kill tumor cells, then return the stem cells to “rescue” the bone marrow. The purpose is intensive tumor control with marrow recovery. The mechanism is repopulating the blood system after high-dose treatment. Risks include serious infections and organ toxicity, so it is only done in specialized centers. [17]
Surgeries
1. Craniotomy for gross total tumor resection
This is the main surgery where the neurosurgeon opens the skull and tries to remove the entire visible tumor. The purpose is to lower tumor load as much as possible, which is strongly linked to better survival in CPC. The procedure uses microscopes and navigation tools to protect healthy brain tissue while cutting out the tumor. Risks include bleeding, infection, and new neurological problems, but in expert hands it is the key first step in treatment. [18]
2. Subtotal resection or debulking
Sometimes the tumor cannot be fully removed safely because it is wrapped around important blood vessels or nerves. In these cases, the surgeon removes as much as possible without causing severe damage. The purpose is to reduce pressure and tumor burden before chemotherapy and radiotherapy. The mechanism is mechanical reduction of tumor mass so other treatments have less disease to control.
3. External ventricular drain (EVD) placement
An EVD is a temporary tube placed into the brain’s ventricles to drain excess cerebrospinal fluid and reduce pressure. It is often used around the time of surgery. The purpose is to stabilize the child and protect the brain from high pressure. The procedure is done in the operating room or intensive care unit under sterile conditions. It allows pressure monitoring and controlled drainage but carries infection risk, so it is used for limited periods.
4. Ventriculoperitoneal (VP) shunt or endoscopic third ventriculostomy (ETV)
If hydrocephalus (fluid build-up) remains, the surgeon may place a VP shunt (a tube from the brain to the abdomen) or perform an ETV (creating a small opening inside the brain to bypass the blockage). The purpose is long-term control of CSF flow and pressure. These procedures protect the brain from ongoing damage due to fluid build-up. Shunts can block or get infected, so long-term follow-up is needed.
5. Biopsy (open or endoscopic)
When the tumor diagnosis is uncertain, a small piece may be removed for pathology instead of doing a big resection at first. The purpose is to get tissue for microscopic and molecular tests (such as WHO grade and TP53 status) before planning major treatment. The mechanism is a limited surgery, often done with endoscopic tools through a small opening, to reduce risk while still obtaining enough tissue to guide therapy.
Preventions
For most families, there is no known way to fully prevent choroid plexus carcinoma, because it is rare and often linked to genetic factors like TP53 mutations that cannot be changed. [19] [2] Prevention focuses on reducing complications and catching problems early:
Early medical review for symptoms – See a doctor quickly for repeated vomiting, worsening early-morning headaches, fast head growth in babies, or new seizures.
Genetic counseling for high-risk families – Families with known Li-Fraumeni or TP53 changes may be offered regular scans and counseling to detect tumors very early.
Safe pregnancy and birth care – Regular antenatal care and avoiding harmful exposures (like tobacco smoke and unnecessary radiation imaging) may help overall brain health.
Avoiding unnecessary radiation exposure – Use MRI instead of CT scans when appropriate, especially in children, to reduce lifetime radiation dose.
Vaccination and infection prevention – Keeping vaccines up to date and using good hand hygiene reduces infection risk during and after treatment.
Adhering to treatment plans – Completing chemotherapy and follow-up visits reduces relapse risk and allows early treatment of complications.
Monitoring for late effects – Regular hearing, vision, hormone, and learning checks catch problems early so they can be treated.
Healthy lifestyle – Balanced diet, enough sleep, and age-appropriate physical activity support recovery and general resilience.
Mental health support – Early help for anxiety or depression can improve cooperation with treatment and follow-up.
Family education – Knowing emergency signs (seizures, sudden severe headache, confusion) and having a clear “when to call” plan with the oncology team helps prevent serious crises.
When to See Doctors
You should contact a doctor or the oncology team immediately (or go to emergency services) if a child with CPC or a history of CPC has:
Sudden, severe, or quickly worsening headaches, especially in the morning or with vomiting.
Repeated vomiting not explained by a stomach bug, especially with sleepiness or behavior changes.
New seizures, jerking movements, staring spells, or sudden loss of awareness.
New weakness, clumsiness, balance problems, double vision, or changes in speech.
High fever, chills, or breathing trouble during or after chemotherapy.
Very low energy, pale skin, fast heartbeat, or bleeding and bruising without clear reason.
Any rapid change that “feels wrong” to parents or caregivers.
For families without a diagnosis but seeing these signs, it is important to see a doctor quickly for assessment and, if needed, brain imaging. [1] [3]
What to Eat and What to Avoid
1. Focus on energy-rich, balanced meals
Offer small, frequent meals with a mix of carbohydrates (rice, bread, potatoes), proteins (egg, fish, lentils, chicken), and healthy fats (vegetable oils, nut butters if safe). This supports growth and healing during and after treatment.
2. Include plenty of fruits and vegetables
Colorful fruits and vegetables give vitamins, minerals, and fiber. Soft, cooked options (mashed carrots, stewed apples, bananas) are easier to eat when appetite is low or mouth is sore.
3. Encourage safe protein sources
Cooked eggs, lean meat, fish, dairy (if tolerated), and beans help repair tissues and support immune cells. Make sure meat and eggs are fully cooked to lower infection risk.
4. Keep the child well hydrated
Offer clean water, oral rehydration solutions, and, if allowed by the team, diluted juices or soups. Good hydration helps kidneys clear chemotherapy drugs and reduce constipation.
5. Avoid raw or undercooked animal products
Skip raw eggs, sushi, rare meat, and unpasteurized milk or cheese, because they can carry germs that are dangerous for children with low immunity.
6. Avoid unwashed raw fruits and salads in high-risk periods
When white cells are very low, some centers recommend avoiding raw salad leaves and unpeeled fruits. Instead, use thoroughly washed, peeled, or cooked options to reduce infection risk.
7. Limit very sugary and highly processed foods
Foods like soft drinks, candy, chips, and instant noodles give calories but little nutrition. They can worsen blood sugar swings and reduce appetite for healthier foods.
8. Be careful with herbal or “immune booster” products
Many herbal mixtures or “natural” tonics can interact with chemotherapy or affect liver function. Always ask the oncology team before using any herbal product. Do not start them on your own.
9. Adjust texture for comfort
If chewing or swallowing is hard, choose soft foods: porridge, yogurt, smoothies, soups, mashed vegetables, and finely chopped meats. This reduces pain and makes eating less tiring.
10. Follow dietitian and oncology advice
Every child is different. The hospital dietitian can design a plan that respects cultural food habits, family budget, and the child’s preferences while staying safe and nutritious.
FAQs
1. Is choroid plexus carcinoma always cancerous?
Yes. Choroid plexus carcinoma is the malignant, cancerous form of choroid plexus tumor (WHO grade 3). There are also benign or less aggressive forms called choroid plexus papilloma (grade 1) and atypical papilloma (grade 2). Carcinoma grows faster, spreads more, and needs stronger treatment. [1] [2]
2. What causes choroid plexus carcinoma?
In many children, the exact cause is unknown. Some cases are linked to inherited changes in a gene called TP53, as seen in Li-Fraumeni syndrome. These changes make it harder for cells to repair DNA damage. Most families did nothing wrong; it is not caused by usual foods or activities.
3. How is choroid plexus carcinoma diagnosed?
Doctors use brain MRI or CT scans to see the tumor and look for hydrocephalus. Surgery or biopsy gives tissue for the pathologist to examine under a microscope and with special tests. The tumor is then graded and sometimes tested for TP53 and other markers.
4. Is surgery always needed?
Almost always, yes. Surgery to remove as much of the tumor as safely possible is the main first step. The amount removed is very important for outcome. However, in some situations, only partial removal or biopsy is safe, and other treatments must do more of the work.
5. Why is chemotherapy used after surgery?
Even after a “total” removal, tiny tumor cells can remain in the brain or CSF. Chemotherapy drugs travel in the blood (and sometimes CSF) to kill these hidden cells. This reduces the chance of the tumor coming back and can sometimes allow lower doses or delayed use of radiotherapy in very young children. [3]
6. Will my child always need radiotherapy?
Not always. Many treatment plans for CPC use both chemotherapy and radiotherapy, especially for older children or when the tumor has spread. Very young children may receive intensive chemotherapy to delay or sometimes avoid radiotherapy because radiation can affect brain development. Decisions are very individual and taken by the tumor board.
7. Can choroid plexus carcinoma spread to other parts of the brain or spine?
Yes. CPC can spread through the cerebrospinal fluid to other areas along the brain and spinal cord. This is called leptomeningeal spread. That is why MRI of the brain and spine and sometimes CSF tests are done at diagnosis and during follow-up.
8. What is the prognosis (outlook)?
Outlook depends on many factors: the child’s age, how much of the tumor is removed, whether it has spread, and the tumor’s genetic features (for example, TP53 status). With modern surgery and intensive therapy in expert centers, some children can be cured, but CPC is still a serious disease and needs strong treatment. [2] [4]
9. Will treatment affect my child’s learning and development?
It can. The tumor itself, surgery, chemotherapy, and radiotherapy can all affect thinking, memory, attention, and school performance. This is why early neuropsychology assessment, school support, and rehabilitation are so important, and why doctors try to balance cure with long-term quality of life.
10. Can my child go to school during treatment?
Often, yes, but it may need adjustments. Some children attend school part-time between treatment cycles; others learn at home or in hospital classrooms. The oncology team and school should work together to plan safe attendance, infection precautions, and academic support.
11. Will my child lose their hair?
Many chemotherapy drugs used in CPC cause temporary hair loss. Hair usually grows back after treatment, though sometimes with a different texture or color. Radiotherapy to the head can also affect hair growth in treated areas.
12. Can CPC come back after treatment?
Yes, relapse can happen, especially if the tumor was not completely removed or had high-risk features. Regular follow-up scans and clinic visits are essential. If the tumor comes back, the team may recommend more surgery, chemotherapy, radiotherapy, or high-dose chemotherapy with stem cell rescue.
13. Is there anything parents can do to improve outcomes?
Parents cannot control the biology of the tumor, but they play a huge role by keeping appointments, giving medicines exactly as prescribed, watching for warning signs, supporting good nutrition and hygiene, and working closely with the care team. Emotional support and advocacy at school also matter.
14. Are clinical trials available?
Because CPC is rare, clinical trials and international treatment protocols are very important. In many regions, children may be offered enrollment in trials that test new combinations of surgery, chemotherapy, and radiotherapy, or new ways to protect brain development. Families can ask their team about available trials or registries. [5] [6]
15. Where can families find reliable information and support?
Trusted sources include major cancer centers, national cancer institutes, and recognized brain tumor charities. These organizations provide simple-language information, support groups, and help lines. The oncology team can recommend websites and local groups. Families should avoid websites that promise miracle cures or suggest stopping standard treatments without doctor guidance.
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: January 14, 2026.
