Choroid plexus tumor is a rare growth that starts in the tissue that makes cerebrospinal fluid in the brain. It can cause headaches, nausea, vomiting, and balance problems from pressure in the head. Early symptoms of choroid plexus tumor in infants may include a rapidly enlarging head and irritability. It is most often found in young children, but it can occur at any age. Treatment usually involves surgery, and some people also need chemotherapy or radiation, and outlook depends on the tumor type and how fully it can be removed.
Overview
Choroid plexus tumorSymptoms
Choroid plexus tumor symptoms usually stem from blocked cerebrospinal fluid and raised pressure in the brain. Early symptoms include headaches, morning vomiting, vision changes, and balance problems. Babies may show fast head growth, a bulging soft spot, or seizures.
Outlook and prognosis
Many people with choroid plexus tumor do well, especially when the tumor is found early and fully removed. Outcomes vary by tumor type (papilloma versus carcinoma), size, spread, and age. Ongoing MRI follow‑up and tailored therapies help sustain long‑term health.
Causes and risk factors
The cause of choroid plexus tumor is often unknown; most cases are sporadic. Risk is higher in infants and young children and in families with inherited TP53 mutations (Li-Fraumeni syndrome). No proven lifestyle or environmental risk factors are established.
Genetic influences
Genetics play a meaningful role in choroid plexus tumor. Some tumors arise with inherited changes, especially in TP53 (Li-Fraumeni syndrome), while others have tumor-only mutations. Genetic testing can guide surveillance, treatment choices, and family risk counseling.
Diagnosis
Doctors diagnose choroid plexus tumor using brain MRI or CT to spot an intraventricular mass, often with hydrocephalus. Definitive diagnosis comes from surgical removal or biopsy and pathology. Spine MRI and cerebrospinal fluid tests check for spread.
Treatment and drugs
Treatment for choroid plexus tumor often combines surgery to remove as much tumor as safely possible with targeted therapies. Many benefit from chemotherapy, and some receive carefully planned radiation, especially if complete removal isn’t feasible. Care teams also manage fluid buildup, seizures, or headaches to improve daily comfort.
Symptoms
Pressure inside the head can build when the brain’s normal fluid can’t flow or drains too slowly, leading to headaches, nausea, and changes in vision or balance. These early symptoms of Choroid plexus tumor often come from extra fluid or the growth pressing on nearby brain areas. Symptoms vary from person to person and can change over time. In infants and toddlers, signs can look different than in older children or adults, such as a quickly enlarging head, irritability, or poor feeding.
A deep, pressure-like headache is common when fluid builds up inside the head. It may feel worse in the morning or when coughing or bending. Pain can ease for a while after vomiting.
Repeated morning nausea or vomiting can happen with rising pressure in the brain. It’s a frequent early sign of a choroid plexus tumor. Many notice relief after vomiting, then symptoms return.
Blurry or double vision can appear if pressure affects the nerves that control eye movement. You might notice brief spells of seeing two of things or trouble focusing. Light sensitivity and headaches around the eyes can occur.
Feeling unsteady, clumsy, or dizzy can point to pressure or irritation in areas that control coordination. Walking may feel wide-based or hesitant. Falls or dropping objects may happen more often.
How people usually first notice Choroid plexus tumor?
Many families first notice something is wrong when a child develops persistent headaches, morning nausea or vomiting, unusual sleepiness, or a rapidly growing head size in infants, prompting an urgent check-up. Doctors may pick up early clues such as bulging fontanelle, irritability, or eyes that seem “sunset” downward, and brain imaging then reveals a mass in the choroid plexus causing excess cerebrospinal fluid and pressure; these are often the first signs of choroid plexus tumor. Sometimes the tumor is first spotted on imaging done for repeated “spells,” seizures, or delays in development, especially in babies and young children.
Types of Choroid plexus tumor
Choroid plexus tumor has a few well-recognized variants that differ in how fast they grow and how likely they are to spread within the fluid spaces of the brain and spinal cord. These variants are based on how the cells look under the microscope and how they behave. People may notice different sets of symptoms depending on their situation. Understanding the main types of choroid plexus tumor can help make sense of early symptoms of choroid plexus tumor and guide treatment choices.
Choroid plexus papilloma
This is the most common and typically slow-growing type. It can cause symptoms from fluid buildup in the brain, like headaches, nausea, or balance issues.
Atypical papilloma
This variant grows a bit faster and looks slightly more abnormal under the microscope. Symptoms are similar to papilloma but may progress sooner if pressure in the brain rises.
Choroid plexus carcinoma
This is the aggressive, cancerous type that can spread through cerebrospinal fluid. It often causes more rapid symptom changes and may need combined treatments like surgery and chemotherapy.
What genetics can tell you about symptoms
Some people with choroid plexus tumors linked to TP53 (Li-Fraumeni syndrome) develop headaches, vomiting, or balance problems earlier in childhood because the tumor grows faster and increases pressure in the skull. In contrast, tumors without TP53 changes may progress more slowly, delaying noticeable symptoms.
Causes and risk factors
Most choroid plexus tumors appear by chance when cells in the choroid plexus start growing abnormally. A small share are linked to inherited changes in a tumor suppressor gene called TP53, as seen in Li-Fraumeni syndrome. Researchers are still clarifying the causes of choroid plexus tumor, and there are few proven environmental or lifestyle triggers. Prior head radiation may raise general brain tumor risk, and these tumors occur more often in infants and young children. Some risks are modifiable (things you can change), others are non-modifiable (things you can’t).
Environmental and biological risk factors
Most families never get a clear answer for why a choroid plexus tumor develops. Some risks are carried inside the body, others come from the world around us. Because this tumor is rare, what we know about risk often comes from broader research on childhood brain tumors. Here’s what current evidence supports.
Most choroid plexus tumors occur in infants and young children. Rapid brain growth and cell turnover during this time may increase vulnerability to tumor change. Adults can be affected, but it is less common.
Past high-dose radiation to the head, usually from medical treatment, increases the risk of several brain tumors. For a rare tumor like this, direct data are limited. Clinicians aim to avoid unnecessary radiation exposure, especially in children.
Significant radiation exposure during pregnancy has been linked with higher rates of childhood brain tumors. For this tumor specifically, evidence is sparse, but nonurgent radiation-based imaging is typically avoided during pregnancy.
No consistent link has been proven between common chemical exposures and this tumor. Research is ongoing, and no specific chemical has enough evidence to be called a clear risk.
Genetic risk factors
Genetic factors play a meaningful role in who develops a choroid plexus tumor, especially in children. Some people inherit a higher chance through specific syndromes or gene changes, while others have tumor changes that arise only in the growth itself. Carrying a genetic change doesn’t guarantee the condition will appear. Knowing which pattern is present can guide genetic counseling, testing, and follow-up plans.
Li-Fraumeni syndrome: An inherited change in the TP53 gene greatly raises the risk of choroid plexus carcinoma in childhood. Families may be offered genetic testing and regular brain imaging based on age and history. Clinicians may also review early symptoms of choroid plexus tumor so care can move quickly if concerns arise.
Aicardi syndrome: This rare, mostly female genetic condition has been linked to choroid plexus papillomas more often than expected. If present, doctors may coordinate neurologic care and consider targeted imaging when symptoms change.
TP53 tumor changes: Many choroid plexus carcinomas carry TP53 mutations in the tumor cells that drive growth. These are usually not inherited and do not raise risk for relatives.
Chromosomal alterations: Choroid plexus tumors often show extra or missing pieces of chromosomes in the tumor itself. These patterns can help confirm the diagnosis and guide classification.
Familial clustering: Rare families have more than one member with a choroid plexus tumor, sometimes explained by TP53 changes. When this occurs without a known syndrome, genetic counseling can help decide if testing is appropriate.
Lifestyle risk factors
Lifestyle habits do not cause choroid plexus tumor, but they can influence symptoms, treatment tolerance, and recovery. This overview explains how lifestyle affects Choroid plexus tumor and highlights lifestyle risk factors for Choroid plexus tumor. Choices around activity, diet, sleep, and substance use can affect headaches, seizures, infection risk, and healing. Always individualize changes with your neurosurgery and oncology team.
Gentle, regular movement improves fatigue, balance, and functional recovery after surgery or chemotherapy. It also lowers blood clot risk and can reduce deconditioning during treatment.
A balanced, protein-rich diet supports wound healing and immune function during therapy. Limiting added sugars helps manage steroid-related weight gain and high blood sugar.
Consistent fluid intake may reduce headache triggers and constipation from pain medicines. Your team may tailor fluids if there are concerns about intracranial pressure or sodium balance.
Adequate, regular sleep can lessen headaches and support cognition after brain surgery or treatment. Sleep deprivation can increase seizure risk in people with brain tumors.
Risk prevention
Choroid plexus tumor is rare, and there’s no proven way to fully prevent it. Risk reduction focuses on genetic risk assessment, careful use of imaging that avoids unnecessary radiation, and finding concerning signs early—especially in infants and young children. Some families benefit from special screening if a hereditary cancer syndrome is present. Knowing your risks can guide which preventive steps matter most.
If close relatives had choroid plexus tumor or Li-Fraumeni syndrome (TP53), talk with a genetics professional about testing. Positive results may lead to regular brain MRI screening and tailored plans for you or your child.
People with confirmed TP53 changes often follow a surveillance plan that can include periodic brain MRI. Early checks may help find tumors at a smaller, more treatable stage.
Avoid unnecessary head CT scans, especially in children, and ask if MRI or ultrasound could answer the question instead. Lowering exposure to ionizing radiation may reduce overall brain tumor risk.
Regular pediatric visits help track head size, development, and neurologic signs. Subtle changes can trigger earlier imaging and referral.
Transmission
Choroid plexus tumor is not contagious; it cannot be passed from one person to another through touch, coughing, or everyday contact. Most choroid plexus tumors are not inherited and arise by chance, but a minority happen in families with an underlying cancer risk condition such as Li-Fraumeni syndrome, where a parent can pass down a gene change that raises risk. Sometimes the gene change is new in the child, so there may be no family history. If cancer runs in the family or a child has unusually early cancers, a genetics professional can explain how choroid plexus tumor is inherited and whether genetic testing makes sense.
When to test your genes
Test your genes if a choroid plexus tumor is diagnosed, especially in children or if there’s a family history of early cancers. Genetic testing can identify syndromes like Li-Fraumeni (TP53) that change screening and treatment. Ask sooner if tumors are multiple, bilateral, unusually early, or recur.
Diagnosis
For many, the first step comes when everyday activities start feeling harder—morning headaches, nausea, or balance trouble that doesn’t go away. The diagnosis of Choroid plexus tumor usually starts with a careful exam and brain imaging to look for a mass and signs of blocked fluid flow. Doctors usually begin with brain MRI because it shows soft tissues and fluid pathways clearly. From there, tissue testing confirms the exact tumor type and helps guide next steps.
A clinician reviews headaches, vomiting, vision changes, and balance or coordination issues. They check reflexes, strength, eye movements, and signs of pressure inside the skull such as swelling of the optic nerve.
MRI with contrast maps the tumor’s size, location, and how it affects the brain’s fluid pathways. It helps show whether the tumor is causing hydrocephalus and guides surgical planning.
CT can be done quickly if symptoms are urgent or MRI isn’t available. It can show bleeding, fluid buildup, or a mass in the ventricles, though it’s less detailed than MRI.
An ophthalmologist may look for swelling of the optic nerve (papilledema). This finding supports increased pressure and strengthens the case for urgent brain imaging.
Stages of Choroid plexus tumor
Choroid plexus tumor does not have defined progression stages. Brain tumors like this are grouped by tumor type and grade (how the cells look and behave) and by whether cells have spread through the fluid spaces around the brain and spine, rather than by numbered stages. Different tests may be suggested to help confirm and map the tumor, including MRI of the brain (and sometimes the spine), a lumbar puncture to check cerebrospinal fluid, and surgical pathology after removal or biopsy. Early symptoms of choroid plexus tumor—such as headaches, morning vomiting, an enlarging head in infants, or balance changes—are considered alongside exam findings and scans to plan treatment and follow-up.
Outlook and prognosis
Many people ask, “What does this mean for my future?”, and the answer depends on the tumor type, size, and whether it has spread beyond the ventricles. Choroid plexus tumor outlook ranges from very favorable to more guarded. The noncancerous form (choroid plexus papilloma) is often curable with surgery, especially when the surgeon can remove it completely. In contrast, atypical papillomas carry a small but real risk of coming back, and choroid plexus carcinoma is aggressive and may need a mix of surgery, chemotherapy, and sometimes radiation.
Doctors call this the prognosis—a medical word for likely outcomes. For choroid plexus papilloma, five‑year survival is high when the tumor can be fully removed and hydrocephalus is managed. Carcinomas have lower survival rates, which vary by age, how much tumor can be removed, and whether cancer cells have spread through the cerebrospinal fluid. Early care can make a real difference, because controlling pressure in the brain, treating early symptoms of choroid plexus tumor like headaches or vomiting, and planning surgery at a high‑volume center all improve the odds.
Everyone’s journey looks a little different. In children and adults with completely resected papillomas, long‑term quality of life is often good, though some may need follow‑up for learning or balance issues. With carcinomas, long‑term effects from the disease and its treatment are more likely, and there is a higher risk of relapse in the first few years; most recurrences, if they happen, appear within two to three years. Talk with your doctor about what your personal outlook might look like, including survival statistics, the plan for imaging follow‑up, and options if the tumor returns.
Long-term effects
Choroid plexus tumor can leave lasting effects that depend on tumor type, age at diagnosis, and treatments like surgery, radiation, or chemotherapy. Long-term effects vary widely, and some fade with time while others may persist. Day to day, this can mean changes in energy, thinking speed, or balance that shape school, work, and play. Some people notice that symptoms linked to pressure in the brain improve after treatment, but new challenges can appear later.
Many with choroid plexus papilloma do well long term after complete surgery. Atypical papilloma and carcinoma carry higher risks of relapse and complications. Outcomes often reflect how fully the tumor could be removed and whether it spread in the fluid spaces.
The tumor can return months or years later, especially with atypical papilloma or carcinoma. This risk is lower after complete removal of a papilloma. Ongoing imaging is usually needed to watch for change.
Fluid buildup can persist even after treatment and may require a long-term shunt. Shunts can clog or get infected, leading to headaches, vomiting, or lethargy. Some eventually outgrow shunt needs, while others remain shunt-dependent.
Attention, processing speed, and memory can be slower after a choroid plexus tumor and its treatments. Children may notice schoolwork takes longer or that multitasking is harder. Changes can be subtle at first and become clearer as academic demands rise.
How is it to live with Choroid plexus tumor?
Living with a choroid plexus tumor often means moving through cycles of monitoring, treatment, and recovery while managing symptoms like headaches, nausea, or balance changes that can ebb and flow. Daily life may involve regular brain imaging, follow-up visits, and attention to fluid balance because these tumors can disrupt cerebrospinal fluid, sometimes causing hydrocephalus; for many, this affects energy, school or work schedules, and driving or activity safety until symptoms are controlled. Families and friends usually become part of the care circle—helping with transportation, appointments, and emotional support—while also coping with uncertainty and the practical demands of recovery. With a coordinated care team, clear plans, and rehabilitation when needed, many people regain routines and independence, even if life takes on a new rhythm centered on vigilance and self-care.
Treatment and drugs
Treatment for choroid plexus tumor depends on the tumor type (papilloma vs. carcinoma), size, location, age, and whether it has spread. Surgery is the main treatment; the goal is to remove as much of the tumor as safely possible, which often also reduces excess cerebrospinal fluid and pressure symptoms. When complete removal isn’t possible or the tumor is cancerous or has spread, doctors may add chemotherapy and, in selected cases, radiation therapy; a doctor may adjust your dose to balance benefits and side effects. Some people need a temporary drain or a shunt to manage fluid buildup, plus steroids or pain control for short-term symptom relief. Follow-up imaging and visits are important, since what works today may need adjustment later as recovery progresses or if the tumor changes over time.
Non-drug treatment
Headaches, nausea, and trouble with balance can disrupt school, work, and family life when living with choroid plexus tumor. Beyond prescriptions, supportive therapies can ease pressure in the brain, improve function, and reduce stress throughout treatment. Knowing early symptoms of choroid plexus tumor—like morning headaches or vomiting—can prompt faster evaluation and earlier supportive care.
Surgical removal of a choroid plexus tumor aims to take out as much tumor as safely possible. This often lowers pressure and can quickly ease headaches and vomiting. Recovery usually includes close monitoring in the hospital.
An external drain, shunt, or endoscopic third ventriculostomy can relieve hydrocephalus caused by choroid plexus tumor. This lowers intracranial pressure to ease nausea, vision changes, and balance problems. Devices may be temporary or permanent.
Targeted beams can control residual or malignant choroid plexus tumor when surgery alone is not enough. Planning uses MRI and sometimes CT to protect healthy brain tissue. Side effects depend on dose and age.
Proton beams can focus energy more precisely to spare nearby brain structures. It may be considered for children who need radiation. Access depends on location and specialist referral.
Your genes may have an impact on how drugs work for you
Some medicines for choroid plexus tumors work differently from person to person because genes affect how drugs are absorbed, broken down, and targeted in the body. Pharmacogenetic testing can sometimes guide dosing or drug choice to improve effectiveness and reduce side effects.
Pharmacological treatments
Medicines for choroid plexus tumor (CPT) mainly help shrink the tumor with chemotherapy and manage brain pressure, seizures, pain, and nausea around surgery and radiation. Not everyone responds to the same medication in the same way. Medicines can also ease pressure-related headaches, a common early symptom of choroid plexus tumor. Specific drugs and combinations are chosen based on age, tumor type (papilloma versus carcinoma), and what treatments you’ve already had.
Dexamethasone lowers brain swelling and pressure, easing headaches and nausea. Side effects may include trouble sleeping, mood changes, and higher blood sugar; tapering is done slowly to prevent rebound swelling.
Levetiracetam is commonly used; alternatives include valproate or lacosamide if needed. Dosing may be increased or lowered gradually to balance seizure control with side effects.
Acetaminophen can help with headache and post‑procedure pain, and ibuprofen may be used if your team says it’s safe. Short courses of opioids can be considered after surgery; constipation prevention and safety checks are important.
Ondansetron or granisetron often help after surgery and during chemotherapy. Aprepitant (or fosaprepitant) and dexamethasone may be added for platinum‑based regimens to better prevent vomiting.
Genetic influences
Genetics can influence who develops a choroid plexus tumor, but many cases happen without any inherited cause. The strongest known link is a change in the TP53 gene, part of an inherited condition called Li-Fraumeni syndrome, which is especially tied to a more aggressive subtype called choroid plexus carcinoma in children. Having a gene change doesn’t always mean you will develop the condition. Doctors may suggest genetic counseling and testing if a choroid plexus tumor appears at a very young age, if the tumor is the carcinoma type, or if there’s a family history of early cancers. Testing often focuses on TP53 and, when positive, can guide cancer screening and treatment plans for you and close relatives. For most people with a choroid plexus tumor—particularly the papilloma type—the tumor is sporadic, but sharing your family history helps tailor care.
Is Choroid plexus tumor genetic?
Genetics play a meaningful role in choroid plexus tumor. Some tumors arise with inherited changes, especially in TP53 (Li-Fraumeni syndrome), while others have tumor-only mutations. Genetic testing can guide surveillance, treatment choices, and family risk counseling.
Pharmacogenetics—how genetics influence drug effects
Genetic findings can shape how doctors treat choroid plexus tumor, from surgery plans to choices about medicines and radiation. Genetic testing for choroid plexus tumor often looks for changes in the tumor itself and for inherited changes such as TP53; if a TP53 change is present (as in Li-Fraumeni syndrome), teams may try to limit radiation to lower the risk of second cancers while still controlling the tumor. On the drug side, research shows some genetic differences can raise the chance of nerve-related side effects from vincristine or hearing loss with platinum medicines like cisplatin and carboplatin, which are sometimes used for choroid plexus tumor. Because of this, some centers monitor closely and may adjust doses or supportive care if someone seems at higher risk, though routine pharmacogenetic testing for these drugs is not yet standard. Genetics is only one factor—age, kidney function, other medicines, and the exact tumor type also guide dosing and drug choice. Talking with your care team about whether any genetic results should influence chemotherapy or radiation plans can help tailor treatment to you.
Interactions with other conditions
Many living with choroid plexus tumor also face problems caused by fluid buildup in the brain (hydrocephalus), such as headaches, vomiting, or vision changes; shunts placed to drain fluid can help but may bring risks like infection that complicate care. Seizures, migraines, or mood changes can occur in the same person, and doctors call it a “comorbidity” when two conditions occur together. In some families, choroid plexus tumor appears as part of an inherited cancer tendency like Li-Fraumeni syndrome, so relatives may have or develop other cancers, and care teams often coordinate tumor treatment with cancer screening. Chemotherapy and steroids used for choroid plexus tumor can lower immunity or raise blood sugar, which may worsen chronic infections or diabetes if those are already present. Because early symptoms of choroid plexus tumor—headaches, nausea, or irritability in infants—overlap with many common conditions, other diagnoses can mask or delay recognition until pressure effects grow. Sharing all diagnoses and medications with your care team helps them align treatments and reduce drug interactions or side effects.
How genes can cause diseases
Humans have more than 20,000 genes, each carrying out one or a few specific functions in the body. One gene instructs the body to digest lactose from milk, another tells the body how to build strong bones and another prevents the body's cells from beginning to multiply uncontrollably and developing into cancer. As all of these genes combined are the building instructions for our body, a defect in one of these genes can have severe health consequences.
Through decades of genetic research, we know the genetic code of any healthy/functional human gene. We have also identified that in certain positions on a gene, some individuals may have a different genetic letter from the one you have. We call these hotspots "Genetic Variations" or "Variants" in short. In many cases, studies have been able to show that having the genetic letter "G" in the position makes you healthy, but having the letter "A" in the same position disrupts the gene function and causes a disease. Genopedia allows you to view these variants in genes and summarizes all that we know from scientific research, which genetic letters (Genotype) have good or bad consequences on your health or on your traits.
History
Throughout history, people have described sudden, severe headaches in children and adults that came with vomiting, blurry vision, or trouble walking—signs we now recognize as pressure building inside the skull. From early theories to modern research, the story of choroid plexus tumor reflects how doctors learned to connect these symptoms with a small structure deep in the brain that helps make cerebrospinal fluid.
First described in the medical literature as uncommon growths found during surgery or autopsy, choroid plexus tumors were initially understood through what they caused rather than what they were. Before brain scans, doctors relied on neurological exams, skull X‑rays, and, when possible, exploratory surgery. Many people—especially infants and young children—presented with an enlarging head, sleepiness, or irritability. Surgeons noticed that removing the mass could rapidly lower pressure, and over time they traced the source to the choroid plexus in the brain’s ventricles.
With the introduction of computed tomography (CT) in the 1970s and magnetic resonance imaging (MRI) in the 1980s, clinicians could finally see these tumors before operating. Imaging showed brightly enhancing lesions in the ventricles and often revealed excess cerebrospinal fluid flow or blockage, explaining why hydrocephalus was so common. Pathologists refined the classification into papilloma (typically slower‑growing), atypical papilloma, and carcinoma (more aggressive), which helped tailor treatment plans and discussions about outlook.
As medical science evolved, pediatric and adult case series clarified who tends to be affected and how differently choroid plexus tumor can behave by age. Pediatric centers reported most cases in children under 2 years, while adult cases, though rarer, were increasingly recognized. Safer anesthesia, better blood management during surgery, and microsurgical techniques improved outcomes, especially important because these tumors can be very vascular and bleed easily.
Advances in genetics added a new layer. Researchers linked some choroid plexus carcinomas to inherited changes in tumor‑suppressor genes such as TP53, particularly in Li‑Fraumeni syndrome. This insight led to genetic counseling and surveillance for families, and to more individualized care. Laboratory studies also began mapping the molecular signals that drive these tumors, opening the door to targeted therapies under study.
In recent decades, awareness has grown that treatment is not one‑size‑fits‑all. Complete surgical removal remains the cornerstone when feasible, but the roles of chemotherapy and radiation are now guided by tumor type, age, and long‑term side effects. Multidisciplinary teams—neurosurgeons, pediatric and adult neuro‑oncologists, neuroradiologists, pathologists, and genetic counselors—became standard in specialized centers.
Today, the history of choroid plexus tumor shows steady progress: from recognizing patterns of raised pressure, to pinpointing a ventricular source, to classifying types under the microscope, and finally to understanding genetic risks in some families. Each step has improved diagnosis, safety in the operating room, and the chances of long‑term recovery, especially for young children who make up many of those diagnosed.