Anaplastic choroid plexus papilloma is a rare, fast-growing brain tumor that starts in the choroid plexus, the tissue inside the brain ventricles that makes cerebrospinal fluid (CSF). “Anaplastic” means the tumor cells look more abnormal and behave more aggressively than a regular choroid plexus papilloma. This tumor can block the normal flow of CSF, causing raised pressure inside the skull, headaches, vomiting, and other serious symptoms. It is most common in children but can also happen in adults. Treatment usually needs a team of specialists and often includes surgery, radiotherapy, and sometimes chemotherapy.
Anaplastic choroid plexus papilloma is a very rare brain tumor that starts from the choroid plexus, the soft tissue inside the brain ventricles that makes cerebrospinal fluid (CSF). In older medical papers, this name was used for a papilloma that looks more aggressive under the microscope, with fast-dividing, abnormal cells. Today, doctors more often say “atypical choroid plexus papilloma” (WHO grade 2) or “choroid plexus carcinoma” (WHO grade 3) for these higher-grade tumors.
Because this tumor is rare, there are no medicines approved only for “anaplastic choroid plexus papilloma.” Doctors use treatments that are proven in other choroid plexus tumors and high-grade brain tumors in general. These include maximal safe surgical removal, radiation, and chemotherapy drugs such as carboplatin, etoposide, cisplatin, cyclophosphamide, and vincristine, often in combinations tested in international studies.
These tumors can block the flow of CSF or make too much CSF. This raises pressure inside the skull and causes many of the symptoms, such as headache, vomiting, and vision problems. Anaplastic or atypical tumors grow faster and are more likely to spread along the CSF than simple (grade 1) papillomas, so they are treated and followed more closely.
Because they are very rare, most of what we know about anaplastic choroid plexus papilloma comes from case reports and from studies of all choroid plexus tumors together. Doctors use this combined information to guide diagnosis, treatment, and follow-up for patients with this tumor.
Other names
Doctors and researchers may use different names that are close to, or overlap with, “anaplastic choroid plexus papilloma.” These names usually describe a tumor of the choroid plexus with papillary (finger-like) structure but more aggressive, “anaplastic” features.
Common or related terms include:
Atypical choroid plexus papilloma (aCPP, WHO grade 2)
Malignant choroid plexus papilloma (older term, less used now)
Choroid plexus papilloma with anaplastic features
High-grade choroid plexus tumor with papillary pattern
Choroid plexus carcinoma (CPC, WHO grade 3) when the papillary tumor shows full malignant change
These names can be confusing. Pathologists now try to fit tumors clearly into WHO grade 1 (CPP), grade 2 (atypical CPP), or grade 3 (CPC) based on standard microscopic rules, instead of using many overlapping labels.
Types
Because “anaplastic choroid plexus papilloma” is not a formal modern WHO category, it is easier to think in terms of related WHO types that behave like “anaplastic” tumors:
Atypical choroid plexus papilloma (WHO grade 2)
This tumor still looks like a papilloma, but the cells divide more often, and there may be more crowding and mild atypia. It behaves more aggressively than a simple (grade 1) papilloma, with a higher chance of recurrence or spread, and is often what older texts meant by “anaplastic papilloma.”Choroid plexus carcinoma (WHO grade 3)
This is a fully malignant tumor with clear invasion into nearby brain tissue, high cell division, and areas of dead tissue (necrosis). It can still have papillary areas but also solid sheets of tumor cells. Many doctors reserve “anaplastic” for this malignant grade because the cells look very abnormal.Recurrent or progressive choroid plexus papilloma
Sometimes a tumor first diagnosed as simple papilloma later grows back with more aggressive features. When it recurs with higher cell division and atypia, some authors have called it “anaplastic papilloma,” but it is now usually re-classified as atypical CPP or CPC.Intraventricular vs. extraventricular tumors
Most choroid plexus tumors grow inside the ventricles, but rarely they arise in brain tissue near the ventricles. Both locations can show anaplastic features. Location influences symptoms and how easily the tumor can be removed.
Causes
For anaplastic or atypical choroid plexus papilloma, a clear single cause is usually not found. Instead, doctors talk about risk factors and possible mechanisms. Many are based on general choroid plexus tumor research.
Random gene changes in choroid plexus cells
The choroid plexus epithelium can develop random DNA errors as cells copy themselves. Some of these changes may switch on growth signals or switch off “brake” genes, causing uncontrolled growth and anaplastic changes.Changes in developmental genes (OTX2, TRPM3)
Studies suggest that genes like OTX2 and TRPM3, which guide normal choroid plexus development, can be altered in choroid plexus papillomas. When these genes are abnormal, cells may grow in a disordered and more aggressive way.TP53 mutations and Li-Fraumeni syndrome
Some choroid plexus tumors, especially carcinomas, show mutations in the TP53 tumor-suppressor gene and may occur in people with Li-Fraumeni syndrome. This inherited condition makes cells more likely to become malignant, including anaplastic choroid plexus tumors.Von Hippel–Lindau (VHL) syndrome
Choroid plexus tumors can be seen in people with VHL, a genetic disorder affecting blood-vessel-rich tissues. Changes in the VHL gene may promote abnormal blood vessel growth and support tumor formation in the choroid plexus.Down syndrome
Some reports link Down syndrome with a higher chance of certain brain tumors, including choroid plexus tumors. Extra copies of genes on chromosome 21 may change how brain cells grow and repair damage.Aicardi syndrome
Aicardi syndrome, a rare neurodevelopmental disorder, has been associated with choroid plexus tumors. The exact mechanism is unknown, but broad developmental errors in the brain may also affect the choroid plexus and lead to tumor growth.Neurofibromatosis type 2 (NF2)
NF2 affects genes that control cell growth in the nervous system. Although most NF2 tumors are different (like vestibular schwannomas), some patients may develop choroid plexus tumors, suggesting that disrupted growth control can involve this tissue too.Family history of brain tumors
A strong family history of brain tumors may point to shared genetic or environmental risk. Even when no specific syndrome is named, inherited variations in DNA repair or tumor-suppressor genes could make anaplastic changes more likely.Immune system problems
Some sources suggest immune system problems could play a role in choroid plexus papilloma. If immune cells are less effective at recognizing abnormal brain cells, small early tumors may survive and evolve into more aggressive forms.Certain infections (possible but unproven)
Infections that affect the brain or developing fetus might damage DNA or disturb development of the choroid plexus. Evidence is limited, so infections are considered a possible, not proven, factor in tumor formation.Environmental exposures (unknown details)
Doctors suspect that some unknown environmental exposures may contribute, such as radiation or chemicals, but clear links with anaplastic choroid plexus papilloma are not proven. Most patients have no known exposure.Female sex
Choroid plexus tumors seem slightly more common in females in some series. Hormonal or genetic differences between sexes may help explain this small difference, though it does not mean hormones directly cause the tumor.Very young age
Many choroid plexus tumors occur in babies and very young children. During early brain development, cells divide rapidly, and this rapid growth may leave more chances for DNA mistakes that lead to aggressive tumors.General cancer-predisposition syndromes
Any inherited condition that weakens DNA repair or cell-cycle control may increase the risk of choroid plexus tumors. These broad syndromes may not specifically target the choroid plexus but make many tumor types, including anaplastic variants, more likely.Somatic (non-inherited) copy-number changes
Tumor studies show gains or losses of chromosome segments in choroid plexus tumors. These somatic changes are not inherited but happen in the tumor itself and can drive anaplastic behavior by changing many genes at once.Abnormal blood-vessel growth
Choroid plexus is very rich in blood vessels. Abnormal signals that promote new vessel growth can feed tumor cells and support progression from low-grade papilloma to more anaplastic, higher-grade forms.Previous cranial radiation (rare factor)
In general brain tumor research, past radiation to the brain can increase tumor risk years later. This is rarely reported for choroid plexus tumors, but the same mechanism of radiation-induced DNA damage could, in theory, contribute.Developmental errors in the ventricular system
Because choroid plexus forms early in brain development, any error in how the ventricles and their lining develop could create a patch of cells prone to uncontrolled growth and later anaplastic change.CSF overproduction and local stress
Tumor cells often overproduce CSF. The constant high flow and pressure may add mechanical and chemical stress to surrounding tissue, possibly supporting further mutations and more aggressive behavior over time.Currently unknown factors
For many patients, none of the above factors are found. This shows that there are still unknown genetic and environmental factors involved. Research continues to search for new mutations and pathways in choroid plexus tumors.
Symptoms
Symptoms mostly come from high pressure in the skull (raised intracranial pressure) and from the tumor pressing on nearby brain structures. In babies and small children the signs may look different from adults.
Headache
Headache is common and often worse in the morning or when lying down, because pressure inside the skull rises. Children may hold their head, cry, or be unusually quiet. Older patients may describe a heavy, bursting pain.Nausea and vomiting
High brain pressure can trigger the vomiting center in the brainstem. Patients may vomit without feeling sick to the stomach first, and vomiting may be more common in the morning. This can lead to dehydration and weight loss if not treated.Enlarged head size in infants
In babies, the skull bones are not yet fused, so rising pressure can make the head grow faster than normal. Caregivers may notice a rapidly increasing head circumference or a bulging soft spot (fontanelle) on top of the head.Irritability and behavior change
Young children may become unusually fussy, irritable, or less playful. Older children and adults may seem withdrawn, sleepy, or show changes in mood and personality. These changes can reflect discomfort from high brain pressure.Blurred or double vision
Increased pressure can swell the optic nerves and disturb eye movement. Patients may complain of blurry vision, double vision, or difficulty focusing. Eye doctors may see swelling of the optic disc, called papilledema.Seizures
Tumor tissue can irritate nearby brain cells and create abnormal electrical activity. This can cause seizures, which may look like staring spells, limb jerking, or full-body convulsions, depending on the brain area involved.Weakness in arms or legs
If the tumor presses on motor pathways, patients may notice weakness, clumsiness, or dropping objects. They may trip easily or have trouble climbing stairs, reflecting involvement of motor tracts or cerebellum.Balance and coordination problems
Many choroid plexus tumors lie near structures that help control balance. Patients may stagger when walking or have trouble with precise movements like buttoning clothes, showing cerebellar or vestibular involvement.Tiredness and sleepiness
Constant high pressure and poor CSF flow can make people feel very tired. They may sleep more than usual, have low energy, or seem “hard to wake,” which can be an important warning sign of worsening pressure.Poor feeding and weight loss in infants
Babies with high intracranial pressure may feed poorly, spit up more, or lose weight. Caregivers may notice that the baby seems uncomfortable when lying flat or during feeding.Developmental delay or regression
When tumor or pressure affects growing brain regions, a child may reach milestones late or lose skills they already had, such as walking or speaking clearly. This can be a sign of significant brain involvement.Neck pain or stiffness
If the tumor or spread of cells affects lower brain areas or spinal CSF flow, patients may complain of neck pain or stiffness. This can also happen when hydrocephalus stretches the tissues around the brainstem.Hearing or speech problems
Depending on location, tumor pressure can affect areas for hearing and speech. Patients may have trouble understanding language, speaking clearly, or may feel that sounds are muffled or distorted.Endocrine or hormonal changes (rare)
If the tumor or raised pressure affects nearby hypothalamic or pituitary structures, there may be changes in growth, puberty, or other hormone-controlled functions. This is less common but possible with large or long-standing tumors.Symptoms from spread along CSF
Anaplastic tumors can seed tumor cells along the CSF pathways down the spine. This can cause back pain, leg weakness, or new bladder and bowel problems when spinal cord or nerve roots are involved.
Diagnostic tests
Doctors use a set of tests together to diagnose anaplastic choroid plexus papilloma: clinical examination, imaging of the brain and spine, and tissue study under the microscope. Because this tumor is so rare, diagnosis is usually confirmed by a specialist neuropathologist.
Below are 20 important diagnostic tests, grouped into physical exam, manual bedside tests, lab and pathology tests, electrodiagnostic tests, and imaging tests. Many patients will not need every single test; doctors choose based on symptoms and safety.
Physical examination tests
Full neurological examination
The doctor checks alertness, memory (in older patients), eye movements, facial strength, limb strength, sensation, reflexes, and coordination. Patterns of abnormal findings help localize where in the brain the problem is and whether pressure is high.Head size and fontanelle check in infants
In babies, the doctor measures head circumference and feels the soft spot on top of the head. A large head or bulging fontanelle suggests raised intracranial pressure or hydrocephalus, which are common with choroid plexus tumors.General physical exam and vital signs
The doctor checks blood pressure, heart rate, breathing, temperature, weight, and growth pattern, and looks for signs of genetic syndromes (such as unusual facial features or birthmarks) that may be linked with choroid plexus tumors.
Manual bedside neurologic tests
Strength testing of arms and legs
The doctor asks the patient to push and pull against resistance or, in small children, observes how well they move their limbs. Weakness on one side, or in both legs, can suggest tumor pressure on motor pathways.Coordination and balance tests
Older children and adults may be asked to touch their nose with a finger, slide a heel down the opposite shin, or walk heel-to-toe. Trouble with these tasks can indicate cerebellar or brainstem involvement from the tumor or hydrocephalus.Eye and vision checks at the bedside
Simple tests of eye movement, pupil reaction to light, and visual fields can show raised pressure or nerve damage. The doctor may also ask about double vision or use a light to look at the back of the eye for papilledema.
Lab and pathological tests
Basic blood tests (CBC and chemistry)
A complete blood count and blood chemistry panel help check general health, look for anemia or infection, and make sure the patient is ready for surgery or anesthesia. These tests do not diagnose the tumor, but they guide safe treatment.Tests for inherited cancer syndromes
When there is a family history or clinical signs of a syndrome such as Li-Fraumeni or VHL, doctors may order genetic blood tests. Finding a germline mutation can confirm a syndrome and change how the whole family is followed over time.CSF analysis (carefully selected cases)
Cerebrospinal fluid may be taken by lumbar puncture to look for tumor cells or infection. However, this is only done when it is safe, because removing CSF can be dangerous in patients with very high intracranial pressure.Histopathology of resected tumor
The most important test is looking at the tumor tissue removed during surgery. Under the microscope, the pathologist checks papillary structure, cell shape, mitotic rate, necrosis, and brain invasion to decide if it is CPP, atypical CPP, or carcinoma.Immunohistochemistry panel
Special stains highlight proteins that help confirm choroid plexus origin (for example, cytokeratins, transthyretin) and help rule out other papillary tumors that might have spread from outside the brain. Patterns of staining also give prognostic clues.Molecular testing of tumor tissue
Modern centers may analyze tumor DNA and RNA to look for specific mutations or gene expression patterns. Changes in TP53, OTX2, or other pathways can help classify the tumor and may influence future targeted therapies or clinical trial choice.
Electrodiagnostic tests
Electroencephalogram (EEG)
If the patient has seizures or unusual spells, an EEG records the brain’s electrical activity through scalp electrodes. It cannot diagnose the tumor type, but it can show abnormal areas and help guide seizure treatment and safety planning.Evoked potential studies (selected cases)
In some centers, visual or somatosensory evoked potentials are used to assess how signals travel along nerve pathways. They can be used before or during surgery to monitor the function of pathways near the tumor and reduce the risk of damage.
Imaging tests
Cranial ultrasound in infants
In babies with open fontanelles, a simple ultrasound through the soft spot can show enlarged ventricles and sometimes a mass in the ventricle. It is quick and does not use radiation, so it is useful as a first imaging test.CT scan of the head
A CT scan uses X-rays to create pictures of the brain. Choroid plexus tumors often appear as enhancing masses inside the ventricles and may show calcification or bleeding. CT is fast and useful in emergencies to detect hydrocephalus.MRI of the brain with contrast
MRI is the key imaging test. It shows the tumor’s size, exact location, relation to brain structures, and effect on CSF flow. Contrast dye helps outline the tumor. MRI can suggest a choroid plexus tumor but cannot by itself tell the exact grade.MRI of the whole spine
Because anaplastic tumors can spread through CSF, doctors often image the spine as well. New nodules or “drop metastases” along the spinal canal can change the tumor stage and influence radiation or chemotherapy plans.Advanced MRI techniques (perfusion, spectroscopy)
Some centers use perfusion MRI to assess blood flow in the tumor and MR spectroscopy to look at its chemical profile. High blood flow and certain metabolite patterns can support the diagnosis of a high-grade, anaplastic lesion.Post-treatment surveillance MRI
After surgery, and during or after chemo-radiation, repeated MRI scans are used to watch for tumor regrowth or new spread. Early detection of recurrence is especially important in atypical or anaplastic tumors with higher relapse risk.
Non-Pharmacological Treatments (Therapies and Other Approaches)
1. Maximal Safe Surgical Removal (Tumor Resection)
Surgery is usually the main treatment. The neurosurgeon opens the skull, reaches the ventricle, and tries to remove all visible tumor (called gross total resection) while protecting healthy brain tissue. Removing the tumor reduces pressure inside the skull and can greatly improve symptoms and survival. In many choroid plexus tumors, complete removal gives the best chance for long-term control or cure.
2. CSF Diversion (External Ventricular Drain or Shunt)
If the tumor blocks CSF flow and pressure is very high, doctors may place a temporary drain or a permanent shunt. A thin tube moves extra fluid from the brain to a bag or to another body area like the abdomen. This lowers pressure, relieves headaches and vomiting, and protects the brain while other treatments are planned.
3. Radiotherapy (Focused Radiation Treatment)
After surgery, high-energy X-rays can be aimed at the tumor area. Radiation damages tumor cell DNA so they cannot keep dividing. It is usually used when the tumor is anaplastic, when some tumor remains after surgery, or when the tumor spreads. The radiation team carefully plans the dose to target the tumor and protect healthy brain tissue as much as possible.
4. Proton Beam Therapy
In some centers, proton therapy is used instead of standard X-ray radiation. Protons can deposit most of their energy directly in the tumor and less in normal tissue beyond it. This may help reduce long-term side effects in children, such as problems with learning or hormone balance, while still controlling the tumor.
5. Physical Therapy (Physiotherapy)
After brain surgery and other treatments, children and adults may have weakness, balance problems, or trouble walking. Physical therapists use stretching, strength exercises, balance training, and walking practice to rebuild muscle strength and coordination. The goal is to help the person safely return to daily activities like walking, playing, or working, and to prevent long-term disability.
6. Occupational Therapy
Occupational therapists focus on daily tasks such as dressing, writing, using a computer, or eating independently. They may teach simple tricks, give special tools, or suggest changes at home or school. This helps the person stay as independent as possible and reduces caregiver stress, especially after demanding brain surgery and radiotherapy.
7. Speech and Language Therapy
Some patients develop speech, language, or swallowing problems due to the tumor or its treatment. Speech therapists assess how the patient speaks, understands, and swallows. They use targeted exercises and strategies to improve clear speech, safe swallowing, and communication. This can greatly improve quality of life and social interaction.
8. Neurocognitive Rehabilitation
Brain tumors and radiation can affect memory, attention, and learning, especially in children. Neuropsychologists and therapists test thinking skills and design training programs to support memory, school performance, and problem-solving. They also work with teachers to adjust school tasks, so children can continue learning as well as possible.
9. Psychological Counseling and Family Support
A brain tumor diagnosis is extremely stressful. Psychologists and counselors help patients and families manage fear, sadness, and uncertainty. They may teach coping skills, relaxation methods, and ways to talk about the illness. Supportive counseling can reduce anxiety and depression and help families make complex treatment decisions more calmly.
10. Social Work and Practical Support
Social workers help with school or work letters, disability paperwork, financial aid, and transport to hospital. They help families connect with community resources and support groups. This reduces the practical burden of long treatment and allows caregivers to focus more on the patient’s emotional and physical needs.
11. Nutritional Counseling
Dietitians look at the patient’s weight, growth, and lab tests. They suggest a high-protein, nutrient-dense diet to support healing and help manage side effects like nausea or constipation. They may recommend special drinks, textures, or feeding methods so the body gets enough calories and vitamins during intense therapy.
12. Seizure Safety Education
Some patients develop seizures because of the tumor or surgery. Education includes how to recognize seizures, stay safe during an episode, and when to call emergency services. Families learn about avoiding triggers, taking medicines regularly, and protecting the head during a seizure. This reduces injury risk and fear.
13. Pain Management and Palliative Care Support
Palliative care teams focus on comfort and quality of life at all stages. They help control headache, post-surgical pain, nausea, sleep problems, and mood symptoms. This team works alongside oncologists, not instead of them. Their aim is to make treatment more tolerable and life more meaningful, even during tough therapies.
14. School Reintegration Programs
Children with brain tumors may miss many school days. Hospital-school programs and home tutoring help them keep up with lessons. Teachers can adjust workload, give extra time, or change seating for better focus. This support lowers the impact of treatment on education and social development.
15. Physiologic Monitoring and Regular MRI Follow-Up
Regular MRI scans help doctors watch for tumor regrowth or spread. Neurological exams track strength, balance, vision, and memory. Early detection of changes allows quick treatment adjustments, which can improve long-term outcomes and reduce complications from growing tumor tissue.
16. Infection Prevention Practices
Because some treatments lower blood counts, infection risk rises. Non-drug measures include handwashing, masking in crowded areas, good oral care, and avoiding contact with sick people. These simple steps lower the chance of serious infections when the immune system is weak.
17. Hormone and Endocrine Monitoring
Radiation near brain areas that control hormones can cause growth or thyroid problems. Regular blood tests and endocrinology visits help detect these changes early. Non-drug strategies like sleep hygiene, exercise, and healthy diet support hormone balance alongside medical care.
18. Genetic Counseling (Especially for TP53/Li-Fraumeni Syndrome)
Some choroid plexus tumors are linked to inherited TP53 gene changes (Li-Fraumeni syndrome). Genetic counseling explains what this means for the patient and family, discusses testing, and suggests screening for other cancers. This helps families plan for long-term health and make informed choices.
19. Participation in Clinical Trials
Because this tumor is rare, clinical trials are very important. They may test new chemotherapy combinations, targeted therapies, or immunotherapies. Joining a trial can give access to cutting-edge treatments and also helps researchers improve care for future patients with the same rare disease.
20. Spiritual and Peer Support Programs
Many families find comfort in spiritual care, religious leaders, or hospital chaplains. Peer support groups, both in person and online, let families talk with others facing similar diagnoses. Sharing experiences can reduce loneliness, increase hope, and provide practical tips for coping with daily challenges.
Drug Treatments
Important note: No drug is approved only for “anaplastic choroid plexus papilloma.” Doctors usually adapt regimens used for other choroid plexus carcinomas and high-grade brain tumors. Actual drugs, doses, and schedules must be chosen by a specialist oncology team.
Below are key examples of medicines commonly used in choroid plexus tumor regimens or supportive care, with information based on FDA prescribing documents and brain tumor studies.
1. Carboplatin (Platinum Chemotherapy)
Carboplatin is a platinum-based chemotherapy that damages tumor cell DNA so they cannot repair themselves or divide. It is usually given by IV infusion and dosed using kidney function and body size, often every 3–4 weeks as part of a combination regimen. Common side effects include low blood counts, nausea, and risk of infection. It is widely used in choroid plexus tumor protocols and other childhood brain tumors.
2. Etoposide (Topoisomerase II Inhibitor)
Etoposide blocks an enzyme called topoisomerase II, which tumor cells need to unwind DNA during division. It is given IV or by mouth, typically in cycles combined with other drugs like carboplatin or cisplatin. Side effects include low white cells, hair loss, and nausea. Etoposide is one of the most frequently used drugs in choroid plexus carcinoma regimens and related protocols.
3. Cisplatin (Platinum Chemotherapy)
Cisplatin is another platinum drug that forms DNA cross-links, leading to cell death. It is given IV with strong anti-nausea medicines and close kidney and hearing monitoring. It may be part of “Head Start” or ICE-type regimens in aggressive choroid plexus tumors. Key toxicities include hearing damage, kidney injury, and low blood counts.
4. Cyclophosphamide (Alkylating Agent)
Cyclophosphamide adds alkyl groups to DNA, stopping tumor cells from dividing. It is used IV or orally in cycles. Doctors carefully hydrate patients and may use a bladder-protecting drug to reduce bleeding risk. Side effects include low blood counts, hair loss, and nausea. It is part of some regimens for pediatric brain tumors, including choroid plexus carcinomas.
5. Ifosfamide (Alkylating Agent)
Ifosfamide works similarly to cyclophosphamide and is used in ICE protocols (ifosfamide, carboplatin, etoposide) for aggressive brain tumors. It is given IV with hydration and mesna to protect the bladder. Side effects include confusion, kidney problems, and blood count drops. Studies suggest ICE-type regimens can benefit choroid plexus carcinoma and similar tumors.
6. Vincristine (Vinca Alkaloid)
Vincristine blocks microtubules, structures needed for cells to divide. It is given IV weekly or in cycles with other drugs. Main toxicities are nerve damage (causing weakness, foot drop, or constipation) and low blood counts. Vincristine is part of many childhood brain tumor protocols and was included in major choroid plexus tumor studies.
7. High-Dose Methotrexate (Antimetabolite)
Methotrexate blocks folate metabolism, which cells use to build DNA. In high doses, it may be used in “Head Start” protocols for pediatric brain tumors. It requires special rescue medicine (leucovorin), careful fluid management, and close lab monitoring. Side effects include mouth sores, liver irritation, and blood count suppression.
8. Temozolomide (Oral Alkylating Agent)
Temozolomide is taken by mouth and converts to a compound that methylates DNA in tumor cells. It is often used with radiotherapy in other brain tumors, and some centers may use it off-label in aggressive choroid plexus tumors when standard regimens are not suitable. Side effects include fatigue, low blood counts, and nausea.
9. Dexamethasone (Corticosteroid)
Dexamethasone is a powerful steroid that reduces brain swelling around the tumor. It is usually given IV or orally in short courses before or after surgery or during radiation. It can quickly improve headaches, nausea, and weakness caused by swelling. Side effects with longer use include high blood sugar, muscle weakness, and mood changes.
10. Mannitol and Hypertonic Saline (Osmotic Agents)
These IV medicines draw fluid out of brain tissue into the bloodstream, reducing pressure inside the skull. They are used in emergency situations or during surgery to control dangerous swelling. Side effects can include electrolyte imbalances and kidney strain, so careful monitoring is needed.
11. Antiseizure Medicines (e.g., Levetiracetam)
If a patient has seizures, drugs like levetiracetam may be used to stabilize brain electrical activity. They help prevent further seizures and protect the brain from repeated injury. Side effects can include tiredness, mood change, or dizziness.
12. Antiemetics (e.g., Ondansetron)
Chemotherapy and brain pressure can cause severe nausea and vomiting. Antiemetic drugs block certain receptors in the gut and brain to reduce these symptoms. Better nausea control lets patients eat and drink enough and improves their ability to continue treatment.
13. Proton Pump Inhibitors (e.g., Omeprazole)
When steroids and stress irritate the stomach, proton pump inhibitors can protect the stomach lining and lower acid production. This reduces pain, heartburn, and risk of bleeding. They are often used while patients receive high-dose steroids or chemotherapy.
14. Pain Medicines (Acetaminophen, Opioids if Needed)
Headaches and post-surgery pain are common. Simple medicines like acetaminophen may help mild pain, while stronger painkillers may be needed for a short time after surgery. Pain control allows better sleep, feeding, and rehabilitation participation.
15–20. Other Supportive Oncology Medicines
Many other medicines may be used depending on the patient’s needs, such as antibiotics for infections, laxatives for constipation, sleep aids, and anxiety medicines. These do not treat the tumor itself but support the body and mind so that the patient can safely complete surgery, radiation, and chemotherapy.
Dietary Molecular Supplements (Supportive, Not Curative)
Always discuss supplements with the oncology team. Some may interfere with chemotherapy or other medicines.
1. Vitamin D
Vitamin D supports bone health, immune function, and muscle strength. Many children with chronic illness or limited sun exposure have low levels. A typical supplement dose is chosen based on blood tests and age. The doctor adjusts the dose to correct deficiency without causing high calcium levels.
2. Omega-3 Fatty Acids (Fish Oil or Algal Oil)
Omega-3s may reduce inflammation and support heart and brain health. They can be given as capsules or liquid. Typical doses are based on weight and tolerance. Possible side effects include a fishy taste or mild stomach upset. They should be stopped before surgery if there is concern about bleeding risk.
3. High-Protein Oral Nutritional Supplements
Protein shakes or powders can help patients who cannot eat enough solid food. They provide calories, protein, vitamins, and minerals in a small volume. Dietitians choose the right product and dose depending on age, kidney function, and overall nutrition status.
4. Probiotics (With Caution)
Probiotics are “good bacteria” that may support gut health, especially during antibiotics. However, in patients with very low immune cells, probiotics may rarely cause infection. The oncology team decides if they are safe and suggests doses and specific strains.
5. Multivitamin Without High Doses of Antioxidants
A simple daily multivitamin can cover small nutrient gaps caused by poor appetite. Doctors often avoid extremely high antioxidant doses during chemotherapy because they might reduce treatment effectiveness. Doses usually follow age-appropriate daily recommendations.
6. Fiber Supplements (e.g., Psyllium)
Constipation is common due to pain medicines and reduced mobility. Fiber supplements help form soft, bulky stools and improve bowel movements. Adequate fluid intake is essential for fiber to work safely.
7. Calcium and Magnesium Supplements
These minerals support bones and muscles. Certain chemotherapy or steroids can weaken bones or lower magnesium. Blood tests guide whether supplements are needed and in which dose, to avoid kidney stones or abnormal heart rhythms.
8. Oral Rehydration Solutions (Electrolyte Drinks)
Specialized electrolyte drinks replace water and salts lost through vomiting or diarrhea. They help prevent dehydration, low blood pressure, and kidney damage. Doses depend on weight and fluid losses.
9. Glutamine (Under Medical Advice)
Glutamine is an amino acid sometimes studied for reducing mouth sores from chemotherapy. Evidence is mixed, and it may not be suitable for all patients. If used, the oncology team will decide the correct form and dose.
10. Vitamin B Complex
B vitamins support nerve function and energy metabolism. Some chemotherapy drugs can affect nerves, so maintaining adequate B vitamin intake may help overall nerve health, although it does not replace medical treatment. Doses should not greatly exceed usual daily needs unless prescribed.
Drugs for Immunity Support / Regenerative and Stem-Cell–Related Care
These medicines do not treat the tumor itself but help protect the bone marrow and immune system during chemotherapy.
1. Filgrastim-Type Medicines (G-CSF, Daily Injections)
Filgrastim-like drugs are synthetic versions of a natural growth factor that tells the bone marrow to make more white blood cells. They are usually given by daily injection after chemotherapy. This shortens the time of low white cells and reduces infection risk. Common side effects include bone pain and mild fevers.
2. Pegfilgrastim-Type Medicines (Long-Acting G-CSF)
Pegfilgrastim products work like filgrastim but last longer, so they can often be given as a single injection per chemotherapy cycle. They help the white count recover and can reduce hospitalizations due to infections. Side effects are similar to filgrastim, including bone pain and rare allergic reactions.
3. GM-CSF (Sargramostim-Type Medicines)
Granulocyte-macrophage colony-stimulating factor encourages the bone marrow to make several types of white cells. It may be used after very intensive treatments or stem cell collection. Side effects include fevers, bone pain, and injection-site reactions. Doses are calculated by weight and adjusted according to blood counts.
4. Erythropoiesis-Stimulating Agents (e.g., Epoetin-Type Medicines)
These drugs mimic the hormone erythropoietin, which stimulates red blood cell production. They may be considered for severe chemotherapy-related anemia in certain settings. They are given by injection, with doses adjusted by blood tests. Risks include high blood pressure and blood clots, so they must be used carefully.
5. Myeloprotective Drug (e.g., Trilaciclib-Type Medicine)
Trilaciclib is a CDK4/6 inhibitor used in some cancers to protect bone marrow from chemotherapy-induced damage when given before certain regimens. It is given by IV infusion before chemotherapy and may reduce the depth and duration of low blood counts. Its exact use in rare brain tumors is not standard and would typically be in a clinical trial setting.
6. Hematopoietic Stem Cell Mobilization Agents (e.g., Plerixafor)
In very high-risk or relapsed cases, doctors may collect a patient’s own stem cells for intensive chemotherapy and later reinfusion. Drugs like plerixafor, combined with G-CSF, help move stem cells from the bone marrow into the bloodstream so they can be collected. Side effects can include bone pain and mild GI symptoms.
Surgical Procedures
1. Craniotomy with Gross Total Resection
This is the main surgery for anaplastic choroid plexus papilloma. The surgeon opens the skull and fully exposes the ventricle to remove as much tumor as safely possible. The goal is to remove all visible tumor because complete removal is linked with the best long-term control and survival whenever possible.
2. Subtotal Resection (Partial Removal)
Sometimes the tumor is wrapped around important blood vessels or brain structures, making complete removal unsafe. In that case, the surgeon removes as much tumor as possible without causing severe damage. Later, radiotherapy or chemotherapy may be used to control the remaining tumor tissue.
3. Ventriculoperitoneal (VP) Shunt Placement
If hydrocephalus (excess CSF) persists, a VP shunt is placed. A tube connects the brain ventricle to the abdomen, where fluid is absorbed. This surgery is done to control chronic high pressure, prevent headaches and vomiting, and protect vision and brain function.
4. Endoscopic Third Ventriculostomy (ETV) in Selected Cases
Sometimes an endoscopic approach can create a small opening in the floor of the third ventricle, allowing CSF to bypass a blockage. This may reduce or remove the need for a shunt in certain patients, depending on anatomy and tumor location.
5. Reoperation for Recurrence or Residual Tumor
If the tumor grows back or a large remnant progresses, surgeons may perform another operation. The decision is based on the tumor’s growth pattern, the patient’s condition, and imaging. Reoperation may improve survival and relieve renewed symptoms from mass effect.
Preventions (What Can and Cannot Be Prevented)
Because anaplastic choroid plexus papilloma is rare and often arises without a clear cause, there is no known way to fully prevent it. However, patients and families can reduce complications and improve outcomes by:
Following All Scheduled MRI and Clinic Visits – Early detection of recurrence or treatment late-effects allows quicker, more effective interventions.
Managing Infections Quickly – Contact the care team promptly for fever or signs of infection, especially during chemotherapy or after surgery.
Protecting the Head from Injury – Use helmets for biking and take fall-prevention measures to avoid additional brain injury.
Avoiding Smoking and Second-Hand Smoke – For older patients and caregivers, smoke-free homes support overall brain and vascular health.
Keeping Vaccinations Up to Date (as Advised) – Vaccines, timed with the oncology team’s guidance, reduce serious infection risk.
Maintaining Good Nutrition and Hydration – A well-balanced diet supports the immune system and recovery.
Regular Physical Activity as Tolerated – Gentle exercise improves strength, mood, and circulation, which may help recovery and reduce complications like blood clots.
Monitoring Hormones and Growth in Children – Early treatment of hormone or growth problems may prevent serious long-term effects.
Genetic Counseling if TP53 or Other Syndromes Are Suspected – This may guide cancer screening for family members and allow earlier detection of other cancers.
Psychological and Social Support – Reducing chronic stress improves treatment adherence, sleep, and overall health, which indirectly helps prevent complications.
When to See Doctors (Urgent and Routine)
You should seek urgent medical help or emergency care if you notice:
Sudden, severe headache, especially with vomiting or confusion
New seizures or worsening seizure control
Sudden weakness, loss of vision, or trouble speaking
High fever, chills, or feeling very unwell during chemotherapy
Stiff neck, severe drowsiness, or behavior changes
You should see the oncology or neurosurgery team soon (within days) if:
Headaches, nausea, or balance problems slowly get worse
School performance or attention drops without a clear reason
There are new hormone signs, such as very slow growth or extreme tiredness
You notice drainage from the surgical scar, redness, or swelling
You should keep regular follow-up appointments for MRI scans, blood tests, hearing checks, and hormone checks as advised, even if you feel well. These visits are essential to catch tumor recurrence or treatment side effects early.
What to Eat and What to Avoid
What to Eat (Examples)
Fruits and Vegetables – Colorful fruits and vegetables provide vitamins, minerals, and fiber that support immunity and bowel health.
Lean Proteins – Foods like fish, eggs, beans, and lean meat help repair tissues after surgery and during chemotherapy.
Whole Grains – Brown rice, oats, and whole-grain bread give steady energy and fiber to prevent constipation.
Healthy Fats – Olive oil, nuts, seeds, and avocados provide calories and essential fatty acids when appetite is low.
Adequate Fluids – Water and oral rehydration drinks help protect the kidneys and prevent dehydration, especially during vomiting or diarrhea.
What to Avoid or Limit
- Raw or Undercooked Meat, Eggs, and Fish – These carry higher infection risk, which is dangerous when white blood cell counts are low.
- Unpasteurized Milk or Juices – These can contain harmful germs; pasteurized products are safer.
- Very Sugary Drinks and Sweets – They give quick calories but little nutrition and can worsen blood sugar control, especially with steroid use.
- Highly Processed, Salty Snacks – Too much salt can worsen swelling and blood pressure; processed foods often offer few useful nutrients.
- Herbal Supplements Without Medical Advice – Some herbs can interfere with chemotherapy or increase bleeding; always check with the oncology team first.
Frequently Asked Questions (FAQs)
1. Is anaplastic choroid plexus papilloma cancer?
It is considered a malignant, or cancerous, form of choroid plexus tumor because the cells look very abnormal and can grow quickly or spread. However, with modern surgery and carefully planned treatments, many patients can live for years, especially if the tumor can be mostly removed.
2. How is this different from a regular choroid plexus papilloma?
A regular papilloma is usually low-grade and slower-growing. Anaplastic papilloma shows more abnormal cells, more cell division, and more aggressive behavior under the microscope. This means doctors often recommend stronger treatments such as radiation or chemotherapy, not just surgery.
3. Can surgery alone cure this tumor?
In some cases where the tumor is completely removed and there is no spread, surgery may control the disease for a long time. However, because anaplastic tumors are more aggressive, doctors often add radiation and sometimes chemotherapy to lower the chance of recurrence.
4. Why are platinum and etoposide-based regimens often used?
Studies in choroid plexus carcinomas and similar tumors show that combinations including carboplatin or cisplatin, with etoposide and other drugs, can shrink tumors and improve survival. These regimens are therefore adapted for aggressive choroid plexus tumors like anaplastic papillomas.
5. Will my child always need radiation?
Not always. In very young children, doctors may try to avoid or delay radiation because of long-term side effects on the developing brain. They may use more chemotherapy instead. The need for radiation depends on age, how much tumor remains, and whether it has spread.
6. What are the main long-term side effects to watch for?
Possible long-term issues include learning or memory problems, hearing loss, hormonal changes (affecting growth or puberty), and second tumors many years later, especially if there is a TP53 gene problem. Regular follow-up helps catch these problems early.
7. Is this tumor linked to genetics?
Some choroid plexus tumors are linked to inherited TP53 gene changes (Li-Fraumeni syndrome). If suspected, genetic counseling and testing may be recommended. This helps plan cancer screening and discuss family risks.
8. Can lifestyle changes cure the tumor?
No. Healthy eating, exercise, and stress management are important for overall health but cannot replace surgery, radiation, or chemotherapy. They are supportive tools that help the body cope with medical treatments.
9. How often will MRI scans be needed?
At first, MRIs are usually done every few months to look for recurrence. Over time, if the tumor remains controlled, the gap between scans may be extended. The exact schedule depends on your center’s protocol and the individual case.
10. Can my child go back to school?
Many children return to school during or after treatment with some adjustments. School support, lighter workloads, and neurocognitive rehabilitation can help them keep learning and stay connected with friends.
11. Is pregnancy possible after treatment?
Some chemotherapy and radiation can affect fertility. Before treatment, older children and adults can sometimes consider fertility preservation options. Later in life, pregnancy may still be possible, but this depends on the treatments received and individual health, so specialist counseling is important.
12. Are there new treatments being studied?
Yes. Clinical trials are exploring new chemotherapy combinations, targeted drugs, and immunotherapies, often based on tumor genetic testing. Because anaplastic choroid plexus papilloma is rare, trials may be international and at specialized centers.
13. What is the outlook (prognosis)?
Prognosis depends on age, how much tumor is removed, whether it has spread, and the tumor’s genetic profile. In general, more complete tumor removal and treatment at experienced centers are linked with better outcomes.
14. Should we get a second opinion?
Because this tumor is rare and complex, many families seek a second opinion at a large brain tumor or children’s cancer center. This can confirm the diagnosis, review imaging and pathology, and offer access to clinical trials or advanced technologies like proton therapy.
15. What is the most important thing families can do?
Work closely with a trusted neuro-oncology team, keep all follow-up visits, ask questions until you understand each step, and accept help—from relatives, friends, and support teams. This partnership, plus timely evidence-based treatment, gives the best chance for the safest and most effective care.
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.
