Choroid plexus papilloma is a rare, slow-growing brain tumour that starts in the choroid plexus, the tissue inside the brain’s ventricles that makes cerebrospinal fluid (CSF). It is usually a benign (non-cancer) tumour and is classed as World Health Organization (WHO) grade 1.
Choroid plexus papilloma (CPP) is a rare, usually benign (non-cancer) brain tumor that grows from the choroid plexus, the tissue inside the brain ventricles that makes cerebrospinal fluid (CSF). This tumor is called WHO grade I, which means it tends to grow slowly and does not usually spread like a cancer. CPP often causes too much CSF or blocks its flow, leading to hydrocephalus (high pressure inside the skull). Typical symptoms are headache, vomiting, big head size in babies, irritability, and problems with balance or weakness. The main treatment is surgery to remove the tumor, and if doctors can remove it fully, many patients are cured and live a normal life.
This tumour sits inside the fluid spaces of the brain and often makes too much CSF or blocks its flow. This extra fluid increases pressure inside the skull and can cause hydrocephalus (water on the brain) and many of the symptoms people notice.
Choroid plexus papilloma can happen at any age but is most common in babies and young children. In children it usually grows in the lateral ventricles (side ventricles), while in adults it more often grows in the fourth ventricle at the back of the brain.
Other names
Choroid plexus papilloma is also known by several other names that mean almost the same thing. Doctors may call it “papilloma of the choroid plexus,” “CPP,” or “choroid plexus tumour, grade 1.” All these terms describe a benign tumour made of papillary (finger-like) fronds that arise from the choroid plexus lining inside the ventricles.
Types of choroid plexus papilloma
Doctors often talk about choroid plexus tumours as a family, based on how they look under the microscope and how fast they grow.
Classic choroid plexus papilloma (CPP, WHO grade 1) – This is the usual form. The cells look almost like normal choroid plexus but grow in larger fronds. It is benign and usually curable with complete surgery.
Atypical choroid plexus papilloma (aCPP, WHO grade 2) – This type has some extra cell division (mitoses) and slightly more aggressive behaviour than classic CPP, but is still considered a papilloma and usually behaves better than carcinoma.
Classification by location – Doctors sometimes describe CPPs by where they sit: lateral ventricle CPP (common in children), fourth-ventricle CPP (more common in adults), and rarer third-ventricle or cerebellopontine-angle CPP. Location affects symptoms and surgical approach but not the basic benign nature of CPP.
Part of the “choroid plexus tumour” group – In WHO systems, CPP (grade 1) and aCPP (grade 2) are grouped together and clearly separated from choroid plexus carcinoma (CPC, grade 3), which is malignant. This helps doctors choose treatment and explain prognosis.
Causes of choroid plexus papilloma
For many people, the exact cause of choroid plexus papilloma is unknown. Most cases seem to happen “by chance” when a single cell in the choroid plexus starts to grow in an abnormal way.
Random DNA changes in choroid plexus cells – The most common idea is that CPP begins when random genetic (DNA) errors build up in a choroid plexus epithelial cell, allowing it to grow into a tumour. These changes are usually not inherited and happen during life.
Germline TP53 mutations (Li-Fraumeni syndrome) – Some people with Li-Fraumeni syndrome, a cancer-risk condition caused by inherited TP53 gene mutations, have a higher chance of developing choroid plexus tumours, including papillomas.
Aicardi syndrome association – Aicardi syndrome, a rare brain and eye malformation syndrome, is linked with a higher risk of choroid plexus tumours, including CPP, probably because of underlying developmental gene errors affecting brain structures.
Other hereditary cancer-predisposition syndromes – Studies suggest that some families with choroid plexus tumours carry inherited changes in tumour-suppressor genes, meaning their cells are less able to repair DNA damage, so tumours such as CPP appear more easily.
Viral factors (SV40, BK, JC viruses – possible) – Some research has found pieces of certain viruses (SV40, BK, JC) in choroid plexus tumours, but this does not prove they cause CPP. They may only be associated or present in some tumours.
Embryologic development errors – The choroid plexus forms early in fetal life. Small mistakes in how these cells grow and fold during development may later show up as a papilloma in childhood.
Somatic TP53 mutations in tumour cells – Even without inherited Li-Fraumeni syndrome, some CPPs show mutations in TP53 only in the tumour tissue, which may help the tumour cell avoid normal growth “brakes.”
Other tumour-suppressor and cell-cycle gene changes – Research has identified other gene alterations, such as changes in RB and other cell-cycle regulators, which may allow choroid plexus cells to keep dividing when they should stop.
Chromosomal abnormalities – Some CPPs show gains or losses of whole chromosome pieces, which can disturb the balance of growth-controlling genes and push cells towards tumour behaviour.
Syndromic chromosomal disorders (e.g., 9p duplication) – Rare chromosome duplication syndromes have been reported together with CPP, suggesting that having extra copies of certain genes may raise risk.
Radiation exposure (possible but not proven) – In general, ionising radiation to the brain can increase risk of brain tumours. For CPP specifically, clear evidence is limited, but prior cranial radiation is often considered a general risk factor.
Immune system and inflammation effects – Long-lasting inflammation in the central nervous system may, in theory, create an environment where cells are more likely to become abnormal, though this link is not clearly proven for CPP.
Sex-linked or hormonal factors in early life – CPP is more common in very young children, when growth signals and hormones in the brain are strong. This suggests that growth-signalling pathways may play a role in tumour formation.
Abnormal regulation of CSF-producing cells – Choroid plexus cells are designed to produce CSF. If the genes and proteins that control this function become unbalanced, the same pathways might also make the cell prone to uncontrolled growth.
Environmental factors in pregnancy (uncertain) – Like many childhood brain tumours, researchers have looked at maternal infections, chemicals, or medications, but so far no strong, specific environmental cause for CPP has been confirmed.
Interaction of multiple weak risk factors – For many patients, CPP likely develops from a mix of small genetic susceptibilities and random life events rather than a single strong cause, which is why most cases seem isolated.
Cellular stress and oxidative damage – Long-term stress on cells, including oxidative stress, can damage DNA. In choroid plexus tissue, repeated damage and imperfect repair might slowly push cells toward tumour formation.
Abnormal cell-to-cell signalling in ventricles – The choroid plexus communicates with surrounding brain and CSF by chemical signals. Changes in these signalling pathways could promote abnormal cell survival and growth in some people.
Family history of brain tumours – Having close relatives with brain tumours or known cancer-risk syndromes may slightly raise the chance of CPP, mainly because of shared genes that affect repair and growth pathways.
Still “idiopathic” (no clear cause) in many cases – Even with all these possible factors, most CPPs are still called idiopathic, meaning that doctors cannot find a specific trigger in that person’s history or genes.
Symptoms of choroid plexus papilloma
Most symptoms of CPP come from too much CSF or blockage of CSF flow, which raises pressure inside the skull (hydrocephalus).
Headache – Many patients, especially older children and adults, have frequent or severe headaches that are often worse in the morning or when lying down because pressure inside the skull rises overnight.
Nausea and vomiting – Raised pressure in the brain can stimulate vomiting centres, so people often feel sick and may vomit, sometimes without much warning, especially in the morning.
Irritability or excessive crying in infants – Babies cannot describe headache, so they may cry a lot, be fussy, or have trouble settling, which can be a sign of discomfort from hydrocephalus.
Enlarging head size (macrocephaly) – In very young children whose skull bones are not yet fused, extra fluid makes the head grow faster than normal, and the soft spot (fontanelle) may bulge.
Bulging fontanelle – The soft spot on top of a baby’s head may feel tense or bulging, showing that pressure inside the skull is high.
Balance and walking problems – When CPP is in the fourth ventricle or presses on cerebellar structures, people may have unsteady walking, clumsiness, or trouble standing without support.
Visual problems (blurred or double vision) – Increased intracranial pressure can swell the optic nerve (papilledema) and disturb eye movement, causing blurred vision or double vision.
Seizures – Some patients have seizures because the tumour or high pressure irritates nearby brain tissue or alters electrical activity.
Weakness of an arm or leg – If CPP compresses motor pathways, patients may notice weakness, dragging a leg, or trouble using one side of the body.
Changes in behaviour or school performance – Raised pressure or disruption of frontal lobe connections can cause personality changes, poor attention, or dropping school grades.
Neck stiffness or pain – In some cases, bleeding into the CSF or irritation around the brain can cause stiff neck and discomfort, somewhat like meningitis symptoms.
Sleepiness or reduced alertness – As pressure rises, patients may become drowsy, fall asleep easily, or be harder to wake, which is a warning sign needing urgent care.
Vomiting with relief of headache – Many people notice that after vomiting, their headache temporarily improves, because some CSF and pressure are briefly relieved.
Endocrine or growth issues (rare) – Tumours affecting nearby structures or long-standing pressure can, in some cases, disturb hormonal control, leading to poor growth or puberty problems.
Incidental finding without clear symptoms – Sometimes CPP is found by brain imaging done for another reason, especially in adults, or when symptoms are mild and non-specific.
Diagnostic tests for choroid plexus papilloma
Doctors use a mix of bedside examination and special tests to diagnose CPP and its effects on the brain.
Physical-exam tests
General neurological examination – The doctor checks mental state, movement, sensation, reflexes, and coordination. These findings can show raised pressure or which brain areas are affected, guiding further imaging tests.
Measurement of head size and fontanelle in infants – In babies, the doctor measures head circumference and feels the soft spot. Rapid head growth or a bulging fontanelle suggests hydrocephalus from a possible intraventricular tumour.
Eye and fundus examination – Using an ophthalmoscope, the doctor looks at the optic nerves for swelling (papilledema), which is a key sign of raised intracranial pressure from hydrocephalus.
Gait and balance assessment – Simple tasks such as walking, heel-to-toe walking, and standing with feet together help detect cerebellar dysfunction from tumours in the posterior fossa or fourth ventricle.
Vital signs and general physical exam – Checking blood pressure, heart rate, and overall health helps rule out other causes of headache and vomiting and assess fitness for anaesthesia and surgery.
Manual (bedside) neurological tests
Cranial nerve examination – The doctor tests eye movements, facial muscles, swallowing, and hearing to see if the tumour or pressure is affecting brainstem or cranial-nerve pathways.
Motor strength and reflex testing – Manual testing of muscle power and tendon reflexes can show weakness or increased reflexes on one side, suggesting long-tract involvement from pressure or mass effect.
Sensory and coordination tests – Light touch, pinprick, finger-to-nose, and heel-to-shin tests are used to detect subtle sensory loss or coordination problems linked with CPP in certain brain regions.
Simple cognitive and mental-status exam – Asking questions about time, place, memory, and attention helps reveal confusion or cognitive slowing due to raised pressure or chronic hydrocephalus.
Signs of meningeal irritation – In rare cases with bleeding or severe pressure, doctors may gently check for neck stiffness or pain with certain movements, which can suggest irritation of the coverings of the brain.
Laboratory and pathological tests
Routine blood tests – Basic blood work (full blood count, electrolytes, clotting) does not diagnose CPP directly but is important to look for other causes of symptoms and to prepare safely for surgery and anaesthesia.
Cerebrospinal fluid (CSF) analysis – In selected cases where it is safe, lumbar puncture can analyse CSF pressure and contents. However, if CPP is suspected with high intracranial pressure, lumbar puncture is usually avoided because it can worsen brain herniation risk.
Histopathology of the tumour – After surgical removal, the pathologist examines the tumour under the microscope. Papillary fronds lined by uniform choroid plexus-like cells, with very low mitotic activity, confirm the diagnosis of classic CPP.
Immunohistochemistry and molecular testing – Special stains (e.g., for cytokeratin, transthyretin, S-100) and genetic tests help confirm CPP, separate it from other intraventricular tumours, and sometimes detect TP53 or other gene changes.
Electrodiagnostic tests
Electroencephalography (EEG) – If seizures occur, EEG records brain electrical activity and helps confirm that spells are epileptic and which parts of the brain are most affected, guiding treatment.
Evoked-potential studies (selected cases) – Visual or brainstem evoked potentials can test how well nerve pathways carry signals. They may be used in research or pre-surgical planning, especially when the tumour lies near sensitive tracts.
Imaging tests
Cranial ultrasound in infants – In babies with open fontanelles, ultrasound through the soft spot can show an echogenic (bright) mass inside enlarged ventricles, suggesting a choroid plexus tumour even before more advanced scans.
Computed tomography (CT) of the brain – CT often shows a well-defined, lobulated intraventricular mass that is isodense or hyperdense, with strong contrast enhancement and sometimes fine calcifications, plus signs of hydrocephalus.
Magnetic resonance imaging (MRI) of the brain – MRI gives the clearest picture of CPP. The tumour appears as a vividly enhancing frond-like mass within a ventricle, often with surrounding enlarged ventricles, and helps distinguish CPP from other tumour types.
Advanced MRI / angiographic imaging – Techniques like MR or CT angiography and perfusion imaging can show the very rich blood supply of CPP and help surgeons plan safe removal. They also help differentiate CPP from more malignant choroid plexus carcinoma.
Treatment Goals in Choroid Plexus Papilloma
The first goal is to save life and protect the brain by lowering raised pressure in the skull, treating hydrocephalus, and removing as much tumor as is safely possible. The second goal is to preserve brain function, such as vision, movement, memory, speech, and learning. The third goal is to avoid long-term side effects from surgery, radiotherapy, and chemotherapy, especially in babies and children whose brains are still growing. Sometimes surgery alone is enough; in other cases (atypical CPP or related carcinomas), doctors add radiation and chemotherapy. Decisions depend on tumor size, location, age of the patient, and whether any tumor is left behind after surgery.
Non-Pharmacological Treatments
Below are 20 non-drug treatments and supports. Each one includes a simple description, purpose, and how it works in the body or daily life.
Careful Neurosurgical Observation
Sometimes a very small, slow-growing CPP that is not causing symptoms is watched closely instead of being treated right away. Doctors check the patient regularly with MRI scans and neurological exams. The purpose is to avoid big surgery while still keeping the brain safe. The mechanism is simple: early changes in size or symptoms are picked up on follow-up scans, so treatment can start before serious damage happens.Head Elevation and Positioning
Keeping the head slightly raised (about 30 degrees) and straight can help venous blood drain out of the brain and can lower pressure inside the skull. Nurses in the ICU often use this basic step for patients with brain tumors and hydrocephalus. The purpose is to improve comfort and protect the brain from high pressure. The mechanism is purely physical: gravity helps blood and CSF flow out of the head more easily.Controlled Ventilation in Intensive Care
In very sick patients, doctors may use a ventilator (breathing machine) to control carbon dioxide levels in the blood. Lower CO₂ can cause blood vessels in the brain to narrow slightly and reduce brain blood volume, which may temporarily reduce intracranial pressure. The purpose is short-term brain protection during crises or surgery. The mechanism is physiological: CO₂ levels directly affect the width of brain blood vessels.External Ventricular Drain (EVD)
Before or after tumor surgery, a thin tube can be placed into the ventricles and brought out through the skull to drain CSF into a sterile bag. The purpose is rapid control of hydrocephalus and careful monitoring of intracranial pressure. The mechanism is direct drainage of extra CSF, which gives the brain more space and lowers pressure inside the head.Ventriculoperitoneal (VP) Shunt
If hydrocephalus continues after tumor removal, surgeons may place a VP shunt—a small tube that carries excess CSF from the brain to the belly (peritoneal cavity), where it is absorbed. The purpose is long-term control of CSF pressure and prevention of headaches and vision loss. The mechanism is a permanent bypass route for CSF, which keeps the ventricles from staying enlarged.Cerebrospinal Fluid Lumbar Drain or Taps (Selected Cases)
In some patients with communicating hydrocephalus, periodic lumbar punctures or lumbar drains can temporarily remove CSF from the spinal space. The purpose is short-term relief of pressure when surgery is not yet possible. The mechanism is similar to other drainage methods: physical removal of fluid lowers CSF volume and pressure.Conventional Radiotherapy
For atypical CPP or when complete removal is not possible, doctors may give radiotherapy to the tumor bed or whole brain/spine in selected cases. The purpose is to kill remaining tumor cells and reduce recurrence risk. The mechanism is DNA damage in tumor cells using focused high-energy beams, which slows or stops their growth. This is used carefully in children because of long-term side effects.Stereotactic Radiosurgery (Focused Radiation)
Radiosurgery (for example, Gamma Knife® or LINAC-based systems) delivers a very focused radiation dose to a small target of residual CPP. The purpose is to control tiny or deep tumor pieces that are hard to remove surgically. The mechanism is highly concentrated radiation that damages tumor DNA while limiting dose to nearby normal brain.Physical Therapy (Physiotherapy)
After brain surgery or long illness, many patients have weakness, poor balance, or difficulty walking. Physical therapists use exercises, gait training, and strengthening to rebuild muscle strength and coordination. The purpose is to restore independence and prevent long-term disability. The mechanism is repeated, guided movement that retrains muscles and the nervous system to work together again.Occupational Therapy
Occupational therapists help patients relearn daily activities such as dressing, washing, writing, and using tools or school materials. The purpose is to help the person function in home, school, and work despite any remaining deficits. The mechanism is task-specific practice and adaptation (for example, special grips, modified tools) that lowers effort and increases success in daily life.Speech and Language Therapy
If CPP or its treatment affects speech, swallowing, or understanding, speech-language therapists provide special exercises and strategies. The purpose is to improve communication and safe swallowing. The mechanism involves repeated practice of sounds, words, memory tasks, and safe swallowing techniques to rebuild neural pathways.Neurocognitive Rehabilitation
Some patients have attention, memory, or learning problems after hydrocephalus or surgery. Neuropsychologists and therapists use exercises, computer tasks, and compensatory strategies to improve thinking skills. The purpose is long-term brain health and school success. The mechanism is “brain training,” which encourages surviving networks to reorganize and take over lost functions.Psychological Counseling and Family Support
A brain tumor diagnosis is frightening for the patient and family. Counseling, support groups, and psycho-education help people understand the disease, cope with stress, and manage anxiety or depression. The purpose is emotional stability and better quality of life. The mechanism is talk-based therapy, problem-solving, and coping strategies that reduce emotional distress and improve daily functioning.School and Educational Accommodations
Children with CPP may need extra time on tests, shorter school days, or special education services. The purpose is to keep learning on track while respecting fatigue, headaches, or attention difficulties. The mechanism is environmental change: adjusting workload and expectations so the brain can keep healing while the child still learns.Seizure Safety Planning
Although CPP is not always associated with seizures, any brain tumor can increase seizure risk. Non-drug safety planning includes avoiding heights without supervision, safe bathing, and teaching family how to respond to a seizure. The purpose is injury prevention. The mechanism is risk reduction through environmental safety and emergency planning rather than changing brain activity directly.Nutritional Support and Swallow Therapy
Some patients eat poorly due to nausea, fatigue, or swallowing problems after surgery or treatment. Dietitians and speech therapists help choose safe food textures, adequate calories, and balanced nutrition. The purpose is to prevent weight loss, vitamin problems, and weakness. The mechanism is matching food type and amount to the patient’s swallowing ability and energy needs.Headache Lifestyle Management
Simple habits such as regular sleep, good hydration, reduced screen time, and avoiding bright lights can help people with ongoing headaches after CPP treatment. The purpose is to reduce headache frequency without adding more drugs. The mechanism is removal of common headache triggers and better regulation of body rhythms.Balance and Gait Training Devices
Tools like walkers, canes, orthotics, and balance boards may be used in rehab to support walking safely. The purpose is to prevent falls while the brain and muscles heal. The mechanism combines mechanical support (devices) with sensory feedback to help the brain relearn safe walking patterns.Palliative and Supportive Care Planning
In very complex or advanced cases (for example, when CPP is associated with more aggressive choroid plexus carcinoma), palliative care focuses on symptom relief and quality of life, not just tumor control. The purpose is comfort, dignity, and support for the family. The mechanism is a team-based approach to relieve pain, nausea, anxiety, and spiritual or social distress.Multidisciplinary Tumor Board Review
Many hospitals discuss CPP cases in meetings that include neurosurgeons, neurologists, oncologists, radiologists, and pathologists. The purpose is to choose the safest and most effective plan for that specific person. The mechanism is shared decision-making: different experts look at images, pathology, and patient factors and agree on a combined strategy.
Drug Treatments
Note: Names and dosing ranges below are general and based on FDA labels and brain-tumor practice. Exact dose, schedule, and combinations must always be set by the treating team. Many drugs are used “off-label” for CPP based on experience with other brain tumors.
Dexamethasone (Corticosteroid)
Dexamethasone is a strong steroid used to quickly reduce brain swelling and improve symptoms such as headache, nausea, and weakness before and after surgery. It lowers inflammation around the tumor and decreases leaky blood vessels in the brain, so less fluid seeps into tissues. Typical starting doses for brain swelling are a few milligrams several times per day, then slowly reduced as symptoms improve, but the exact schedule is individualized. Common side effects include high blood sugar, mood changes, infection risk, stomach irritation, and muscle weakness.Mannitol (Intravenous Osmotic Agent)
Mannitol is a sugar alcohol given through a vein to pull water out of the brain and into the blood, which reduces intracranial pressure in emergencies. It is often used around surgery or in acute hydrocephalus. Doses are usually calculated by weight (for example, 0.25–1 g/kg) and given as intermittent infusions while monitoring electrolytes and kidney function. Its purpose is rapid, temporary brain pressure control. Side effects can include dehydration, kidney stress, electrolyte imbalance, and, rarely, fluid overload if not monitored carefully.Levetiracetam (Antiseizure Drug)
Levetiracetam is commonly used to prevent or treat seizures in patients with brain tumors, including CPP. It works by modulating neurotransmitter release at nerve endings, stabilizing electrical activity in the brain. Oral and intravenous forms are available, and dosing is based on body weight and kidney function, often taken twice daily. Side effects can include sleepiness, dizziness, mood changes, and irritability, but it usually has fewer drug interactions than older antiseizure medicines.Carboplatin (Platinum Chemotherapy)
Carboplatin is a platinum-based chemotherapy drug used mainly when CPP behaves aggressively, when there is atypical CPP, or when it is part of regimens for related choroid plexus carcinoma. It links to DNA in tumor cells, causing damage that stops cell division and leads to cell death. Dosing is often calculated from kidney function (AUC-based, e.g., AUC 5–6 every 3–4 weeks) in specialized protocols. Important side effects include low blood counts, nausea, hair loss, and risk of allergic reactions.Etoposide (Topoisomerase II Inhibitor)
Etoposide is a chemotherapy drug that blocks an enzyme called topoisomerase II, which is needed for DNA repair and unwinding in rapidly dividing cells. It is used in some pediatric brain-tumor regimens that include choroid plexus tumors, often together with carboplatin and other agents. Doses may be given daily for several days in cycles, by mouth or through a vein. Major side effects are low white blood cells, infection risk, hair loss, and nausea, so blood counts must be checked regularly.Vincristine (Vinca Alkaloid)
Vincristine comes from the periwinkle plant and stops tumor cells from dividing by blocking microtubules, which are structures needed for pulling chromosomes apart. It is intravenous only and must never be given into the spinal fluid. In brain-tumor protocols, small weekly doses are used for several weeks in a cycle. Side effects include nerve damage (numbness, tingling, weakness), constipation, and hair loss. Its careful use as part of combination chemotherapy can help control aggressive choroid plexus tumors, although CPP is often managed with surgery alone.Cyclophosphamide (Alkylating Agent)
Cyclophosphamide is an older but powerful chemotherapy that adds alkyl groups to DNA, leading to strand breaks and cell death in dividing cells. In pediatric oncology, it is sometimes included in high-risk or recurrent choroid plexus tumor regimens. Dosing varies widely (from moderate to very high doses) and is usually given intravenously with strong hydration and bladder protection. Side effects include low blood counts, nausea, hair loss, bladder irritation, and longer-term risk of fertility problems.Methotrexate (Antimetabolite, High-Dose CNS Therapy)
Methotrexate is an antimetabolite that blocks folate pathways needed for DNA synthesis. In some CNS lymphoma and experimental brain-tumor protocols, high-dose methotrexate is used to reach therapeutic levels in the brain and CSF. For central nervous system–directed therapy, very high mg/m² doses are given with “rescue” using leucovorin to protect normal cells. For CPP, its use is rare and usually reserved for very special situations. Side effects at high doses include severe bone-marrow suppression, mouth sores, liver and kidney toxicity, and risk of infections.Temozolomide (Oral Alkylating Agent)
Temozolomide is an oral chemotherapy drug widely used in glioblastoma and other malignant brain tumors. It works by adding methyl groups to DNA, which leads to tumor cell death if repair mechanisms fail. In choroid plexus tumors, it may be used on a case-by-case basis, especially in more malignant variants, but there is limited direct evidence for typical CPP. It is usually taken once daily for several days in a cycle, with dose based on body surface area. Common side effects are low blood counts, nausea, fatigue, and increased infection risk.Broad-Spectrum Antibiotics (for Post-operative or Shunt Infections)
After neurosurgery or shunt placement, doctors may use broad-spectrum antibiotics if there is concern for infection. These medicines do not treat the CPP itself but protect the brain and shunt hardware from bacteria. The purpose is to prevent or treat meningitis, ventriculitis, or wound infections, which can be life-threatening. The mechanism is direct killing or growth-blocking of bacteria through various pathways such as cell-wall interference or protein synthesis inhibition. Specific drugs and doses depend on local patterns and cultures.Proton Pump Inhibitors (for Stomach Protection)
Drugs like omeprazole or pantoprazole reduce stomach acid production and are used when patients receive high-dose steroids like dexamethasone or NSAIDs. The purpose is to lower the risk of stomach ulcers and bleeding. Their mechanism is blocking the proton pump in stomach parietal cells, which decreases acid secretion. Side effects may include headache, diarrhea, and, with long-term use, low magnesium or vitamin B12 levels.Acetaminophen (Paracetamol, Pain and Fever Control)
Acetaminophen is often the first-line medicine for mild to moderate headache or fever in patients with CPP, especially children. It works mainly in the central nervous system to reduce pain perception and fever set-point without significant anti-inflammatory effect. Doses are based on weight, and daily totals must be limited to avoid liver injury. When used correctly under medical supervision, it is generally safe and helps keep the child comfortable.Opioid Analgesics (Short-Term Severe Pain Control)
In the immediate post-surgical period, stronger pain medicines such as morphine or fentanyl may be used to control severe pain. These drugs act mainly on opioid receptors in the brain and spinal cord to change how pain is felt. The purpose is humane pain relief while the surgical wound heals. Side effects can include sleepiness, constipation, nausea, and, in high doses, slowed breathing, so dosing and monitoring must be very careful.Antiemetics (e.g., Ondansetron)
Ondansetron and related drugs help control nausea and vomiting from raised intracranial pressure, anesthesia, or chemotherapy. They work by blocking serotonin (5-HT₃) receptors in the gut and brain that trigger the vomiting reflex. Doses are usually weight-based and given before chemo or as needed. Side effects include headache, constipation, and, rarely, heart rhythm changes.Laxatives and Stool Softeners (e.g., Docusate, Polyethylene Glycol)
Constipation is very common after brain surgery because of pain medicines, reduced movement, and dehydration. Stool softeners and gentle laxatives help keep bowel movements regular. The mechanism is either drawing water into the stool or helping fat and water mix into it, making it easier to pass. The purpose is comfort and prevention of painful straining or bowel blockage.Electrolyte and Fluid Replacement Solutions
After mannitol, surgery, or vomiting, fluids and electrolytes like sodium and potassium must be monitored and corrected. Intravenous fluids are tailored to the patient’s needs to keep blood pressure and organ function stable. The mechanism is simple: replacing exactly what the body loses to maintain normal cell function.Granulocyte Colony-Stimulating Factor (Filgrastim and Pegfilgrastim)
When strong chemotherapy is used, white blood cells often drop, increasing infection risk. Filgrastim and pegfilgrastim stimulate the bone marrow to make more neutrophils. They are given as injections, usually once per cycle for pegylated forms or daily for filgrastim, with doses based on weight. Side effects can include bone pain, injection-site reactions, and rare spleen problems. These drugs do not treat CPP directly but allow safer delivery of intensive chemotherapy.Prophylactic Anticoagulants (Low-Dose Heparin in Older Patients)
Older adolescents or adults who must stay in bed for long periods may receive small doses of blood thinners to prevent blood clots in the legs or lungs. These drugs act on the clotting cascade to reduce clot formation. The purpose is to prevent deep-vein thrombosis and pulmonary embolism during hospital stays. Side effects mainly involve bleeding, so doctors balance clot risk and bleeding risk very carefully.Immunotherapy Agents (e.g., Pembrolizumab for Associated Malignancies)
In rare complex cases where choroid plexus carcinoma or another cancer is present, immune checkpoint inhibitors such as pembrolizumab may be considered as part of broader cancer care. They work by blocking the PD-1/PD-L1 pathway, “releasing the brakes” on the immune system so it can attack tumor cells. These are not routine for benign CPP but may be relevant in overlapping situations. Side effects can include immune-related inflammation of many organs (skin, gut, liver, lungs, endocrine glands).Targeted or Trial-Based Therapies (In Clinical Trials Only)
Because CPP is rare, some patients may be offered clinical trial drugs targeting specific molecular pathways if the tumor shows certain mutations. These drugs work on exact proteins (for example, growth factor receptors or signaling pathways). The purpose is to improve control in resistant or recurrent disease while limiting damage to normal cells. Mechanism and side effects depend on the specific agent, and these are only given under trial protocols.
Dietary Molecular Supplements
Always check with the treating team before using any supplement. Some can interfere with chemotherapy or other medicines.
Omega-3 Fatty Acids (Fish Oil or Algae Oil)
Omega-3 fats (EPA and DHA) help build cell membranes in the brain and have mild anti-inflammatory effects. In children recovering from CPP treatment, they may support general brain health and mood, though they do not shrink the tumor. Typical doses are modest, based on age and weight, and should be discussed with a doctor. Side effects are usually mild, such as fishy after-taste or stomach upset, but high doses can increase bleeding risk, especially with blood thinners.Vitamin D
Vitamin D is important for bone health, immune function, and possibly mood. Children on steroids or who are indoors for long periods may have low vitamin D, which can weaken bones. Supplement doses are based on blood levels and age, often taken once daily or weekly. The mechanism is improved calcium absorption and bone mineralization. Too much vitamin D can cause high calcium, so supervised dosing is essential.Calcium
Steroids and reduced mobility can weaken bones, so calcium supplements are often paired with vitamin D. Calcium supports healthy bone structure and nerve and muscle function. Doses are usually divided across the day and adjusted for age and diet. Too much calcium can cause kidney stones or constipation, so balance with diet and fluids is important.B-Complex Vitamins (Especially B6, B12, Folate)
B vitamins support energy metabolism and nerve function. After long illness, poor appetite or chemotherapy, some patients may develop deficiencies, which can worsen fatigue and neuropathy. Supplements at standard daily doses can help restore normal levels, but mega-doses are usually not needed. The mechanism is providing necessary cofactors for many metabolic reactions in the brain and body.Probiotics
Probiotic supplements contain beneficial bacteria that may help maintain gut health during and after antibiotics or chemotherapy. They may reduce diarrhea and help restore a balanced gut microbiome, which in turn supports immune function. However, in very immunocompromised patients they must be used cautiously. Dosing is usually expressed in colony-forming units (CFUs) and taken daily.Magnesium
Magnesium is involved in muscle relaxation, nerve function, and energy production. Low magnesium can happen with certain medications or poor nutrition and may cause cramps or fatigue. Supplements can correct deficiency and may slightly improve sleep quality. Too much magnesium, especially in kidney disease, can be dangerous, so medical guidance is needed.Zinc
Zinc plays roles in immune function and wound healing. After neurosurgery, adequate zinc may support normal tissue repair and immune responses. Zinc supplements are usually given in small daily doses and should not exceed recommended limits for age, because very high zinc can disturb copper balance and immune function.Iron (If Iron-Deficiency Anemia Is Present)
If blood tests show iron-deficiency anemia due to poor intake or blood loss, iron supplements help restore hemoglobin and energy levels. They work by providing the raw material the body needs to make red blood cells. Side effects can include stomach upset and constipation, so doses are adjusted carefully and taken with or without food depending on tolerance.Multivitamin for Age Group
A standard, age-appropriate multivitamin may help fill small gaps in diet, especially when appetite is low. It is not a treatment for CPP but helps overall resilience. The mechanism is providing small amounts of many vitamins and minerals that support body functions. Overdosing with multiple overlapping supplements should be avoided.Protein-Rich Oral Nutrition Supplements
In underweight or very tired patients, ready-made drinks or powders with balanced protein, carbohydrates, fats, and micronutrients can support recovery. Protein helps rebuild muscles and immune cells. Doses are usually one to several servings per day, depending on diet and weight. Good hydration and kidney function are important to handle extra protein safely.
Immunity-Booster / Regenerative / Stem-Cell-Related Drugs
These drugs support the body’s ability to recover from treatment; they do not directly “cure” CPP itself. Many are used only in special, high-risk situations.
Filgrastim (G-CSF)
Filgrastim is a lab-made form of granulocyte colony-stimulating factor, a natural hormone that tells bone marrow to make more neutrophils (a type of white blood cell). It is used after chemotherapy to shorten the time of low white-cell counts, reducing infection risk. It is given as a daily injection with dose based on weight. Side effects often include bone pain and mild fever.Pegfilgrastim (Long-Acting G-CSF)
Pegfilgrastim is similar to filgrastim but stays in the body longer because of a pegylated (PEG) chemical chain. It is usually given once per chemotherapy cycle instead of daily injections. The purpose and mechanism are the same: boosting neutrophil production to lower infection risk. Side effects are similar, with bone pain being the most common complaint.Erythropoiesis-Stimulating Agents (e.g., Epoetin Alfa)
In patients with long-term anemia from chemotherapy or chronic illness, drugs like epoetin alfa may be used to stimulate red blood cell production. They mimic erythropoietin, a hormone made by the kidneys. This can improve fatigue and exercise tolerance. However, they must be used carefully because too high a hemoglobin level can increase risk of blood clots.High-Dose Intravenous Immunoglobulin (IVIG) in Special Cases
IVIG is a purified mixture of antibodies from many donors. It is sometimes used when there are complex immune problems or specific antibody deficiencies, though it is not routine for CPP alone. IVIG works by adjusting immune responses and providing ready-made antibodies. Side effects can be headache, fever, or rare kidney issues, so it is given under close supervision.Hematopoietic Stem Cell Support (in Very High-Dose Chemo Protocols)
In rare cases with extremely high-dose chemotherapy, doctors may collect a patient’s own blood-forming stem cells beforehand and give them back later to help rebuild the bone marrow. Drugs like filgrastim are used to move stem cells from marrow into the blood so they can be collected. This process is complex and reserved for aggressive cancers, not typical benign CPP.Emerging Regenerative and Cell-Based Therapies (Clinical Trials Only)
Experimental research is exploring mesenchymal stem cells and other cell-based therapies for brain injury and radiation damage, but these are not established treatments for CPP. Their purpose is to regenerate or protect normal brain tissue after intensive therapy. Mechanisms may involve secretion of growth factors and immune-modulating molecules. Because evidence is still limited, these approaches are only used in research settings with strict oversight.
Surgical Procedures Main Operations and Why They Are Done
Craniotomy with Gross Total Resection of Tumor
This is the main curative treatment for CPP. Surgeons open the skull (craniotomy), find the tumor in the ventricle, carefully control bleeding (CPPs are very vascular), and remove the tumor as completely as possible. The purpose is to eliminate the tumor and stop hydrocephalus caused by CSF over-production. When total removal is achieved, long-term cure rates are very high.Subtotal Resection (Partial Removal) When Total Removal Is Unsafe
If the tumor is stuck to vital brain structures or blood vessels, surgeons may remove only part of it to avoid serious damage. The purpose is to relieve pressure and symptoms while keeping the patient safe. Later, radiotherapy or careful observation may be used for the remaining tumor.Endoscopic or Minimally Invasive Tumor Removal in Selected Cases
In some locations, parts of a CPP can be removed with an endoscope (a thin tube with a camera) through a small opening, sometimes combined with CSF diversion. The purpose is to reduce tumor mass and hydrocephalus with smaller incisions. The mechanism is direct tumor removal under video guidance, limiting disruption of normal brain tissue.Ventriculoperitoneal Shunt Surgery
As described earlier, this surgery places a permanent tube from the ventricle to the abdomen for excess CSF drainage. It is done when hydrocephalus persists even after tumor surgery. The purpose is long-term control of CSF pressure and prevention of future pressure-related symptoms.Endoscopic Third Ventriculostomy (ETV) in Suitable Patients
In some older children and adults, surgeons can make a tiny opening in the floor of the third ventricle to allow CSF to bypass a blockage and flow more normally. The purpose is to treat obstructive hydrocephalus without a permanent shunt. The mechanism is creating an internal “short-cut” for CSF circulation. ETV is not suitable for every CPP case but can be an option in expert centers.
Prevention Strategies
Because CPP is usually not caused by lifestyle, there is no sure way to prevent it. However, you can reduce complications and support recovery with these measures:
Early Evaluation of Persistent Headaches, Vomiting, or Enlarging Head in Babies – Seeing a doctor early when symptoms persist helps detect hydrocephalus or tumors sooner and prevents severe brain pressure.
Regular Follow-Up Imaging After Surgery – Keeping scheduled MRI or CT scans helps find any regrowth early, when treatment is easier and safer.
Protecting the Shunt (If Present) – Avoiding rough pulling on the shunt tubing, watching for redness or swelling along the path, and seeking care if symptoms of shunt failure appear can prevent serious emergencies.
Vaccination and Infection Control – Staying up to date with vaccines and practicing good hand hygiene reduces infection risk, especially in children with shunts or low white-cell counts from chemotherapy.
Healthy Nutrition and Hydration – Balanced meals and adequate fluids support wound healing, immune function, and energy levels during recovery.
Avoiding Head Trauma – Wearing helmets for biking or sports and using seat belts help prevent additional brain injury in someone who has had brain surgery.
Adherence to Medication Plans – Taking seizure medicines, steroids, and other drugs exactly as prescribed lowers the risk of seizures, acute swelling, and other complications.
Regular Eye and Developmental Check-Ups in Children – Monitoring vision, learning, and growth helps detect subtle problems early, so therapies can be started before school or social difficulties build up.
Psychological and Social Support – Joining support groups and mental-health services can reduce stress, which indirectly supports immune function, sleep, and treatment adherence.
Participation in Follow-Up Programs or Tumor Registries – Being part of specialized follow-up programs improves long-term monitoring and can contribute to research that may help future patients.
When to See a Doctor
You should seek urgent medical attention if someone with known or suspected CPP has any of the following:
New or worsening severe headache, especially with early-morning vomiting or neck stiffness.
Sudden change in alertness, confusion, extreme sleepiness, or difficulty waking up.
New seizure or change in seizure pattern.
Sudden weakness, trouble walking, loss of balance, or changes in speech or vision.
Rapid increase in head size or bulging soft spot in an infant.
Signs of shunt malfunction in a child with a VP shunt: headache, vomiting, irritability, or swelling over the shunt path.
Fever with neck stiffness or worsening headache that could suggest infection.
These signs may mean raised intracranial pressure, shunt failure, bleeding, or infection, all of which need fast assessment in an emergency department.
Diet – What to Eat and What to Avoid
Eat Balanced Meals with Enough Protein – Include eggs, fish, beans, or lean meat to help heal the brain and muscles after surgery.
Choose Colorful Fruits and Vegetables – These foods provide vitamins, minerals, and antioxidants that support immune and tissue repair processes.
Stay Well Hydrated – Drinking enough water (as advised by the doctor) helps prevent constipation and supports blood flow and kidney function, especially when taking mannitol or chemotherapy.
Include Whole Grains for Steady Energy – Foods like brown rice, whole-wheat bread, and oats give steady glucose to the brain and help with fiber intake.
Limit Extra Sugar and Highly Processed Snacks – Too many sweet drinks, candy, and fast foods can worsen fatigue and weight gain, especially when taking steroids like dexamethasone.
Moderate Salt Intake – Very salty foods may worsen fluid retention and blood pressure during steroid therapy, so salt should be used carefully.
Avoid Alcohol (for Teens and Adults) – Alcohol can interact with many medicines, affect balance, and worsen liver stress from drugs like acetaminophen or chemotherapy, so it is best avoided.
Be Careful with Herbal Supplements – Herbs like St. John’s wort, ginkgo, or high-dose green tea extracts can interact with chemotherapy and seizure medicines, so they should not be used without specialist advice.
Avoid Raw or Undercooked Animal Products During Chemotherapy – When white cells are low, raw eggs, sushi, or undercooked meats may carry higher infection risk, so foods should be fully cooked.
Tailor Diet to Nausea and Appetite – Small, frequent meals, bland foods, and cold rather than hot dishes may be easier when nausea is present. Working with a dietitian helps match diet to the person’s symptoms and preferences.
Frequently Asked Questions
Is choroid plexus papilloma cancer?
CPP is usually a benign, WHO grade I tumor, which means it does not behave like a classic aggressive cancer. However, it is still serious because it grows inside the brain and causes hydrocephalus and pressure problems. Atypical CPP and choroid plexus carcinoma are more aggressive related tumors.Can CPP be cured?
Many CPPs can be cured if surgeons can remove the entire tumor safely. Large studies show excellent long-term survival when gross total resection is achieved. Recurrences are uncommon but can happen, so long-term follow-up is needed.Why does CPP cause hydrocephalus?
CPP forms in the choroid plexus, which makes CSF. The tumor can either produce too much fluid or block its normal pathways. This leads to buildup of CSF in the ventricles, raising pressure and causing headaches, vomiting, and big head size in infants.Do all patients with CPP need chemotherapy or radiotherapy?
No. Many patients, especially with classic CPP, need only surgery. Chemo and radiotherapy are usually reserved for atypical CPP, residual tumor that cannot be removed safely, recurrence, or related choroid plexus carcinoma.Is CPP more common in children or adults?
CPP occurs at any age but is more common in children, especially in the first years of life, and in the lateral ventricles. In adults it is more often found in the fourth ventricle.What are the main risks of surgery for CPP?
Because CPP is very vascular, blood loss is a major concern, especially in small children. There is also risk of infection, stroke, weakness, speech problems, and seizures. In experienced centers, careful planning and modern techniques keep risks as low as possible.Can CPP come back after surgery?
It can, but this is not very common when the tumor is completely removed. If any small part is left behind, or if the tumor is atypical, recurrence risk is higher, and doctors may recommend closer imaging and sometimes radiotherapy.Does CPP run in families?
Most CPPs are not clearly inherited, but some choroid plexus tumors are linked to genetic cancer-predisposition syndromes such as Li-Fraumeni (TP53 mutations). In those families, several types of tumors can occur. Genetic counseling may be suggested in complex cases.How long does a patient stay in hospital after CPP surgery?
Hospital stay varies widely. Some patients go home after about a week, while others, especially infants with hydrocephalus or complications, may need longer. Time in the ICU right after surgery is often followed by a regular ward stay and then rehab.Will a person with CPP be able to go back to normal school or work?
Many patients, especially those treated early and successfully, go back to school or work with little or no long-term disability. Others may need educational support or workplace adjustments, depending on any residual neurological or cognitive problems.Do steroids like dexamethasone have long-term side effects?
Yes. Long-term or high-dose steroids can cause weight gain, mood swings, high blood sugar, high blood pressure, bone thinning, and muscle weakness. Doctors try to use the lowest effective dose for the shortest time and taper slowly.Can diet or supplements cure CPP?
No diet or supplement can remove or cure a brain tumor. Healthy eating and supplements can support recovery, immune function, and energy, but they must never replace surgery or recommended medical treatments.Are there warning signs of shunt blockage after CPP surgery?
Warning signs include worsening headache, vomiting, irritability or sleepiness, changes in vision or balance, and swelling along the shunt path. Any of these signs need urgent medical review because shunt failure can quickly become life-threatening.How often will follow-up scans be needed?
In general, more frequent scans (for example, every few months) are done in the first years after surgery, then less often if everything stays stable. The exact schedule depends on tumor type, age, and whether any tumor remains.What should families remember most about CPP treatment?
Families should remember that CPP is usually treatable and often curable with proper care in a specialized center. Close follow-up, good communication with the neurosurgery and oncology team, healthy lifestyle habits, and attention to new symptoms are key parts of long-term success.
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.
