Choroid plexus cancer is a rare brain tumor that starts in the fluid‑making tissue inside the brain. People with choroid plexus cancer may have headaches, nausea, vomiting, or balance problems, and babies or toddlers may have a fast‑growing head size. It can grow quickly and needs prompt treatment, and not everyone will have the same experience. Choroid plexus cancer is most often diagnosed in young children, but it can occur at any age. Treatment usually includes surgery and may include chemotherapy and radiation, and survival depends on tumor type, age, and how fully the tumor can be removed.
Overview
Choroid plexus cancerSymptoms
Choroid plexus cancer can raise pressure in the brain, causing headaches, morning vomiting, sleepiness, irritability, and vision changes. Early symptoms of choroid plexus cancer in infants include a fast-growing head or bulging soft spot; seizures or balance problems may appear.
Outlook and prognosis
Most people with choroid plexus cancer do best when it’s found early and fully removed with surgery, often followed by chemo and sometimes radiation. Outcomes vary by tumor grade, age, and how far it has spread. Ongoing follow‑up scans help catch recurrences early.
Causes and risk factors
Choroid plexus cancer usually arises sporadically; its exact cause is unknown. Risk increases in children, especially under age 5, and in families with inherited TP53 variants (Li-Fraumeni syndrome). Prior head radiation may raise risk; lifestyle factors are not established.
Genetic influences
Genetics play a major role in choroid plexus cancer. Some cases arise from inherited syndromes (like Li-Fraumeni) or tumor DNA changes that drive growth. Genetic testing can guide risk assessment, screening for relatives, prognosis, and targeted treatment options.
Diagnosis
Doctors suspect choroid plexus cancer on brain MRI or CT showing a ventricular mass and fluid buildup. Diagnosis of choroid plexus cancer is confirmed by surgical removal or biopsy with pathology. Staging includes spine MRI and cerebrospinal fluid cytology.
Treatment and drugs
Treatment for choroid plexus cancer usually combines surgery to remove as much tumor as safely possible with chemotherapy, and often radiation tailored to age and tumor type. Care may include corticosteroids to reduce swelling and medicines for nausea or pain. Many benefit from coordinated care at centers experienced in pediatric and adult brain tumors, plus rehabilitation and follow-up imaging.
Symptoms
Daily life changes may show up as ongoing headaches, morning nausea, or a child who seems unusually sleepy or irritable. Symptoms vary from person to person and can change over time. Early symptoms of choroid plexus cancer often stem from rising pressure inside the skull due to blocked fluid flow, affecting thinking, movement, and vision.
Headaches that are frequent, worsening, or worse in the morning can happen when pressure inside the head increases. Coughing, bending, or lying flat can make the pain flare. These patterns can occur with choroid plexus cancer if the tumor blocks fluid pathways.
Unexplained morning nausea or vomiting can happen even without a stomach bug. People often feel brief relief after vomiting, but it tends to return if pressure remains high.
Blurry or double vision, brief graying-out, or trouble focusing can appear as pressure affects the visual pathways. These changes can occur in choroid plexus cancer as pressure rises.
Unsteadiness, clumsiness, or veering to one side can develop. You might trip more, drop items, or feel dizzy when turning quickly.
How people usually first notice Choroid plexus cancer?
Many families first notice something is wrong when a child develops sudden, severe symptoms like persistent headaches, repeated vomiting (especially in the morning), unusual sleepiness, or irritability; infants may show a rapidly growing head size or a bulging soft spot. Doctors are often alerted by signs of increased pressure in the skull or developmental changes, and imaging like MRI or CT scans done for these symptoms reveals a mass in the choroid plexus. In newborns and babies, the first signs of choroid plexus cancer are often rapid head growth and poor feeding, while in older children they’re more likely to be headaches, balance problems, or vision changes.
Types of Choroid plexus cancer
Choroid plexus cancer is rare, and doctors recognize a few distinct types based on how the tumor cells look under the microscope and how fast they grow. These types often track with age and risk: young children more often have aggressive forms, while adults more often have slower-growing tumors. Symptoms don’t always look the same for everyone. When people search for types of choroid plexus cancer, they’re usually asking how these subtypes differ in behavior and treatment approach.
Choroid plexus papilloma
This is the least aggressive type, considered benign but still capable of causing symptoms by blocking fluid flow. It often leads to pressure-related symptoms like headaches, vomiting, or larger head size in infants.
Atypical papilloma
This sits between papilloma and carcinoma in behavior, with features suggesting a higher risk of coming back. It may grow faster than a papilloma and can need closer follow-up and additional treatment after surgery.
Choroid plexus carcinoma
This is the malignant, fast-growing type that can spread through the fluid spaces of the brain and spine. It often causes symptoms to progress quickly and may require a combination of surgery, chemotherapy, and sometimes radiation.
RELA-fusion carcinoma
A molecular subtype of choroid plexus carcinoma defined by a RELA gene fusion. It tends to behave more aggressively and is identified with specialized tumor testing.
TP53-altered carcinoma
Another molecular subtype of choroid plexus carcinoma linked to TP53 changes, sometimes associated with Li-Fraumeni syndrome. It can signal higher risk and may prompt genetic counseling and tailored treatment planning.
What genetics can tell you about symptoms
Some choroid plexus cancers are linked to inherited TP53 changes (Li-Fraumeni syndrome), which raise the chance of early, fast-growing tumors that can cause headaches, vomiting, and balance problems. Rare mismatch repair gene variants can also lead to multiple tumors and earlier symptoms.
Causes and risk factors
The exact cause is often unknown, and there are no proven lifestyle risk factors for choroid plexus cancer. Some risks are written in our DNA, passed down through families. People with Li-Fraumeni syndrome from TP53 gene changes have a higher risk of choroid plexus cancer. It is more common in infants and young children. Past high-dose radiation to the head can raise brain tumor risk, but a clear link to choroid plexus cancer is limited.
Environmental and biological risk factors
Choroid plexus cancer is rare, so most families will never face it, but understanding what raises risk can make conversations with your care team clearer. Doctors often group risks into internal (biological) and external (environmental). Below are risk factors for choroid plexus cancer in children and adults that relate to the body or to exposures in the world.
This tumor is most often diagnosed in infants and young children. Age is a biological factor, with risk highest in the first years of life and much lower in adults.
High-dose radiation to the head, especially during childhood, raises the chance of developing brain tumors, including rare types like choroid plexus cancer. Risk tends to increase with higher doses and younger age at exposure. Medical imaging uses far lower doses, but repeated CT scans can add up over time.
Exposure of a fetus to significant ionizing radiation can raise the future risk of childhood brain tumors. This is uncommon and typically linked to high-dose situations such as certain radiation treatments or accidents. Ultrasound does not use radiation.
Genetic risk factors
Genetic changes can play a major role in who develops choroid plexus cancer and when it appears. Some risk factors are inherited through our genes. The strongest inherited link is a change in the TP53 gene, often seen in Li-Fraumeni syndrome; other genetic changes happen only in the tumor itself. Knowing your family pattern and considering genetic counseling can clarify personal risk and guide earlier checks if early symptoms of choroid plexus cancer arise.
TP53 germline variants: An inherited or birth-present change in the TP53 gene is the most established genetic risk for choroid plexus cancer, often as part of Li-Fraumeni syndrome. Children with this change have a much higher chance of developing the tumor, often at younger ages. Rates can vary between families and regions.
TP53 in tumors: Many choroid plexus cancers also acquire TP53 changes only within the tumor cells. These changes drive tumor growth but are not shared by relatives.
Chromosome changes: Tumor cells commonly show large gains and losses of chromosome segments. These shifts can boost growth signals and silence natural brakes on cell division.
Inheritance pattern: If a parent carries a TP53 change, each child has a 50% chance to inherit it. This can lead to cancers across several generations.
De novo variants: In some children, the TP53 change is new and not found in either parent. In those cases, siblings usually have a low chance of carrying the same change.
Family history clues: Multiple relatives with cancers at young ages, including brain tumors or sarcomas, can point to an inherited TP53-related risk. A genetics professional can advise on testing and next steps.
Other rare syndromes: Rarely, tumors occur alongside other inherited cancer syndromes. These links are less consistent than TP53 and can differ by family.
Lifestyle risk factors
Choroid plexus cancer is not caused by lifestyle choices; however, daily habits can shape symptom burden, treatment tolerance, and complication risks. Understanding how lifestyle affects Choroid plexus cancer can help patients and families support care. Below are lifestyle risk factors for Choroid plexus cancer that may worsen or ease the course, depending on how they’re managed.
Eating enough protein and calories supports healing, weight maintenance, and immune function during surgery, chemotherapy, and steroids. Poor intake can worsen fatigue, increase infection risk, and lead to treatment delays.
Gentle, clinician-approved movement can improve fatigue, mood, and balance during and after treatment. Overexertion or contact sports may aggravate headaches and increase risks after craniotomy or shunt placement.
Regular, adequate sleep can ease headaches, stabilize mood, and support attention affected by the tumor or therapies. Irregular or short sleep may worsen daytime fatigue and cognitive problems.
Steady fluid intake can reduce headache triggers, constipation from opioids, and kidney stress from some chemotherapies. Dehydration may intensify nausea, dizziness, and hospitalizations.
Risk prevention
Choroid plexus cancer is rare, and for most people there’s no proven way to prevent the tumor itself. Prevention is about lowering risk, not eliminating it completely. The most effective steps focus on knowing your family history, considering genetic counseling if there’s a pattern of early cancers, and catching any problems early—especially in families with TP53 changes (often called Li‑Fraumeni syndrome). For high‑risk children and adults, plans often include regular imaging and avoiding unnecessary radiation.
Share any brain tumors or early cancers in close relatives. Patterns can point to inherited risks like TP53 changes. This helps your team tailor prevention and screening.
A genetics professional can explain benefits and limits of TP53 testing. Results guide surveillance plans for you and relatives. Counseling also supports informed decisions over time.
If you carry a TP53 variant, periodic brain MRI can help detect tumors sooner. Schedules are individualized by age and history. Imaging plans aim to find problems before symptoms escalate.
Know early symptoms of choroid plexus cancer—persistent morning headaches, repeated vomiting, balance problems, or vision changes. New or worsening symptoms deserve prompt medical review. Early checks can speed diagnosis and treatment.
Transmission
Choroid plexus cancer is not contagious—you can’t catch it or pass it to others through contact, air, or bodily fluids. Most cases happen by chance when cells in the choroid plexus develop harmful gene changes, not because it was picked up from someone else. In a smaller number of families, the risk is inherited, most often due to a change in a gene called TP53; if a parent carries this change, each child has a 50% chance to inherit it, which can raise the risk of choroid plexus cancer and other cancers. New (de novo) gene changes can also appear for the first time in a child, so there may be no prior family history. If you’re wondering how choroid plexus cancer is inherited, a genetics professional can discuss testing and what results may mean for relatives.
When to test your genes
Consider genetic testing soon after diagnosis, or earlier if there’s a strong family history of rare childhood brain tumors, multiple cancers at young ages, or features suggesting a cancer‑predisposition syndrome. Testing can reveal inherited risks (like TP53 mutations in Li-Fraumeni syndrome) that guide surveillance and treatment choices. Ask your oncology team or a genetic counselor now.
Diagnosis
You might notice small changes in daily routines, like morning headaches, nausea, or new balance problems that lead to a medical visit. Getting a diagnosis is often a turning point toward answers and support. Doctors use several steps for the diagnosis of choroid plexus cancer, starting with symptoms and moving to imaging and tissue testing. The goal is to confirm the tumor type and map where it has spread so treatment can be planned safely.
Doctors ask about symptoms such as headaches, vomiting, seizures, or behavior changes and check vision, strength, and balance. Findings help decide which tests to do first and how urgent they are.
MRI creates detailed pictures of the brain to locate the mass, its size, and how it affects nearby fluid spaces. It helps distinguish choroid plexus cancer from other tumors but cannot confirm the exact type on its own.
A head CT can quickly show a mass or fluid buildup if MRI is not immediately available. It is also useful in emergencies to assess pressure or bleeding.
Removing a small tissue sample allows pathologists to confirm the tumor under the microscope. In many cases, surgeons remove as much of the tumor as safely possible and send tissue for testing at the same time.
Stages of Choroid plexus cancer
Choroid plexus cancer does not have defined progression stages. Instead of numbered stages, brain tumors like this are described by where the tumor is, whether cancer cells have spread in the fluid spaces around the brain and spine, and what the tumor tissue looks like under the microscope. Doctors assess based on symptoms—such as early symptoms of choroid plexus cancer like headaches, vomiting, or balance changes—along with MRI scans of the brain (and sometimes spine) and a surgical biopsy to confirm the type. Different tests may be suggested to help confirm the diagnosis, check the fluid around the brain and spinal cord, and track response over time, and some families may be offered genetic counseling.
Outlook and prognosis
Many people ask, “What does this mean for my future?”, and with choroid plexus cancer the answer depends on tumor type, age, and how fully it can be removed. Pediatric cases are more common; when surgeons can take out the entire tumor and follow with the right chemotherapy—and radiation in selected situations—long-term survival improves significantly. In adults, this cancer is rarer and outcomes vary widely, partly because diagnosis may come later or the tumor behaves differently. Doctors call this the prognosis—a medical word for likely outcomes.
Looking at the long-term picture can be helpful. Complete surgical removal is the single strongest predictor of outcome, and early symptoms of choroid plexus cancer—like morning headaches, nausea, or balance changes from fluid buildup—often prompt the scans that make timely surgery possible. High‑grade tumors tend to recur more and usually need multi‑drug chemotherapy and, when safe, radiation; low‑grade tumors relapse less often but still require close follow‑up with MRI. In medical terms, the long-term outlook is often shaped by both genetics and lifestyle.
The outlook is not the same for everyone, but survival has improved over the past two decades at experienced centers, with the best results seen when care is coordinated by pediatric or neuro‑oncology teams. When relapse happens, additional surgery, different chemotherapy combinations, or clinical trials may extend control; palliative care can be added early to ease symptoms without closing doors on treatment. Mortality risk is higher for high‑grade tumors and for cases where the tumor can’t be fully removed, yet many living with choroid plexus cancer achieve years of good quality life after intensive therapy. Talk with your doctor about what your personal outlook might look like.
Long-term effects
Choroid plexus cancer can leave lasting effects that show up months or years after treatment, shaped by the tumor’s location, pressure in the brain, and the therapies used. Long-term effects vary widely, and no two people have the exact same path. Many families first notice early symptoms of choroid plexus cancer like headaches or morning vomiting, but the long-term picture focuses more on thinking skills, movement, senses, and general energy. With regular follow-up, care teams aim to prevent complications, catch changes early, and support school, work, and daily life.
Attention, memory, and processing speed may be slower after choroid plexus cancer. School or work can feel harder, especially with multitasking. Some skills improve over time with therapy.
Weakness, clumsiness, or balance problems can linger, depending on where the tumor and surgery were. Sports or stairs may take extra effort. Physical therapy often helps endurance and coordination.
Some people develop seizures after brain surgery or treatment. Medication can control many seizure disorders. Ongoing follow-up is important if new spells appear.
Headaches may persist, especially with changes in sleep or stress. They can relate to prior brain pressure or treatment effects. New or worsening headaches should be checked promptly.
How is it to live with Choroid plexus cancer?
Living with choroid plexus cancer often means moving through cycles of treatment, recovery, and watchful waiting, with hospital visits becoming part of the weekly routine. Fatigue, headaches, nausea, and changes in thinking or balance can make school, work, and everyday tasks harder, and many find they need help with transportation, childcare, or managing medications. Families and close friends often carry a heavy emotional load while coordinating care, but they also become a powerful support team—advocating at appointments, tracking symptoms, and celebrating small wins. Planning days around energy levels, keeping a simple system for records, and leaning on rehabilitation, counseling, and supportive care can make life more manageable and preserve a sense of normalcy.
Treatment and drugs
Choroid plexus cancer is treated in specialized centers using a mix of surgery, chemotherapy, and sometimes radiation, tailored to the tumor type, location, and a person’s age. Surgeons aim to remove as much of the tumor as safely possible because a more complete removal often improves outcomes, and doctors may place a temporary drain or shunt if fluid buildup (hydrocephalus) causes pressure symptoms. Chemotherapy is commonly used after surgery, and in infants and young children it may be prioritized or combined with lower-dose radiation to protect developing brain tissue. Radiation therapy can target any cancer left behind or that has spread within the fluid spaces, with dosing planned carefully to balance control of the cancer and side effects. Not every treatment works the same way for every person, so care teams adjust the plan over time based on how the cancer responds, any early symptoms of side effects, and the person’s overall recovery.
Non-drug treatment
For choroid plexus cancer, non-drug care focuses on removing the tumor when possible, easing pressure from fluid buildup, and supporting brain recovery. These options work alongside medical treatments and can improve day-to-day function during and after therapy. Non-drug treatments often lay the foundation for long-term quality of life, from surgery and radiation to rehabilitation and school supports.
Surgeons aim to remove as much tumor as safely possible. This can quickly reduce pressure, headaches, and vomiting.
Focused beams target remaining tumor cells when complete removal isn’t possible or if cancer returns. Techniques aim to limit effects on healthy brain tissue, especially in younger children.
This targeted form of radiation can reduce exposure to nearby brain areas. It may lower long-term side effects in some people with choroid plexus cancer.
A temporary drain or permanent shunt can relieve fluid buildup and headaches. This often eases nausea, vision changes, and other pressure symptoms.
Your genes may have an impact on how drugs work for you
Some drugs used for choroid plexus cancer work differently depending on inherited and tumor-specific gene changes that affect how the body processes medicine and how cancer cells resist it. Genetic testing can guide dose, drug choice, and targeted therapies to improve safety and benefit.
Pharmacological treatments
Treatment for choroid plexus cancer often combines surgery with chemotherapy, and medicines are tailored to age, tumor features, and previous treatments. Not everyone responds to the same medication in the same way. Common chemotherapy drugs for choroid plexus cancer include platinum medicines like cisplatin or carboplatin, plus drugs such as etoposide, ifosfamide, cyclophosphamide, vincristine, and methotrexate. In some situations, temozolomide or topotecan may be used, especially if the cancer returns or other options aren’t tolerated.
A core drug in many regimens for aggressive tumors. It can affect hearing and kidneys, so extra hydration and hearing checks are routine.
Often used instead of cisplatin or in combination with other drugs. It commonly lowers blood counts and may be easier on the kidneys and ears than cisplatin, though risks remain.
Frequently paired with platinum drugs to boost cancer cell kill. It can cause low white blood cells and hair thinning, and is given by vein over several days in cycles.
Part of combinations such as ICE (ifosfamide, carboplatin, etoposide). It can irritate the bladder and affect kidneys, so it’s given with protective medicine and close monitoring.
Genetic influences
For some families, choroid plexus cancer is linked to an inherited change in a tumor‑suppressor gene, most often TP53, as part of a condition called Li‑Fraumeni syndrome. In these situations, the protective “brakes” that help keep cell growth in check don’t work as well, which can raise cancer risk. Many cases, however, are not inherited and happen as a new change that appears only in the tumor cells. Family history is one of the strongest clues to a genetic influence. Because of the known link with Li‑Fraumeni, doctors often recommend genetic testing for choroid plexus cancer, especially in children, to check for an inherited TP53 change and to guide follow‑up for relatives. Even when an inherited change is found, it doesn’t mean every family member will develop cancer, and the effect of the same change can differ from person to person.
Is Choroid plexus cancer genetic?
Genetics play a major role in choroid plexus cancer. Some cases arise from inherited syndromes (like Li-Fraumeni) or tumor DNA changes that drive growth. Genetic testing can guide risk assessment, screening for relatives, prognosis, and targeted treatment options.
Pharmacogenetics—how genetics influence drug effects
Genetics can shape how doctors plan treatment for choroid plexus cancer, from the choice of chemotherapy to how and whether radiation is used. Alongside medical history and imaging, genetic testing of the tumor and sometimes your own DNA can guide treatment choices. An inherited change in a gene called TP53 (often linked to Li-Fraumeni syndrome) is found in some people with choroid plexus cancer and can raise the risk of harm from radiation, so teams may favor surgery and chemotherapy and limit radiation when possible. For chemotherapy drugs commonly used in this cancer—such as vincristine, cisplatin, methotrexate, or cyclophosphamide—differences in genes that control how the body breaks down or clears medicine may change the risk of side effects like nerve problems, hearing changes, or low blood counts; in select centers, pharmacogenetic testing may be used to help choose doses or monitoring, but it isn’t standard everywhere. Genetic testing for choroid plexus cancer treatment can also uncover tumor changes that make someone eligible for a clinical trial, though targeted medicines are still being studied in this rare disease. These genetic insights rarely give a black-and-white answer, but they add useful context so your team can personalize therapy based on your age, overall health, and the tumor’s features.
Interactions with other conditions
For some, choroid plexus cancer occurs as part of a hereditary cancer syndrome—most often Li‑Fraumeni—which raises the chance of developing other tumors elsewhere in the body. Talk with your doctor about how your conditions may influence each other. Choroid plexus cancer often causes hydrocephalus (a buildup of fluid in the brain), and the shunts used to relieve pressure can sometimes lead to infections that delay chemotherapy or complicate recovery. If choroid plexus cancer appears alongside inherited syndromes like Li‑Fraumeni—or, less commonly, DICER1‑related conditions—genetic counseling and screening for additional cancers may be recommended for you and close relatives. Treatments can interact too: some chemotherapy drugs can worsen existing kidney or hearing problems, and radiation may be limited in people with TP53‑related syndromes to lower the risk of treatment‑induced cancers. Symptoms from other neurologic conditions—such as epilepsy or migraines—can overlap with early symptoms of choroid plexus cancer like headaches, nausea, or vision changes, so teams often coordinate care to understand what’s coming from where.
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 troubling headaches, vomiting, and changes in alertness in children that seemed to come without warning. Families and communities once noticed patterns of severe illness that moved quickly, often leading doctors to suspect pressure building inside the skull. Looking back helps explain why Choroid plexus cancer was long hard to recognize: its early symptoms can mimic infections, dehydration, or common childhood conditions.
First described in the medical literature as a rare tumor arising from the fluid-making tissue inside the brain’s ventricles, this cancer was initially identified only during surgery or at autopsy. In the late 1800s and early 1900s, doctors recorded cases of infants with rapidly enlarging heads and tense soft spots, signs of fluid build-up rather than a mass itself. Without brain scans, the tumor was invisible; its presence was inferred from the way cerebrospinal fluid could not drain properly.
From these first observations, surgeons began attempting delicate operations to relieve pressure by draining fluid. As X‑rays and then pneumoencephalography appeared, clinicians could outline the brain’s spaces, but not the tumor. Real change arrived with computed tomography (CT) in the 1970s and magnetic resonance imaging (MRI) in the 1980s, which finally showed choroid plexus tumors directly. Over time, descriptions became more precise, separating benign choroid plexus papillomas from malignant choroid plexus carcinomas, and noting that the cancer more often affects very young children but can occur at any age.
In recent decades, awareness has grown around inherited risk. Some children with Choroid plexus cancer have a change in a gene that also raises the chance of other tumors; this led to recommendations for genetic counseling and careful screening of families. Advances in genetics deepened that understanding, linking tumor behavior to specific molecular features and guiding modern treatment studies.
Treatment history followed the tools available. Early care focused on repeat fluid drainage to ease pressure and emergent surgery when possible. With better imaging, neurosurgeons could plan safer, more complete tumor removal. Chemotherapy and, in selected cases, radiation therapy were added, especially when complete removal was not feasible. International collaborations improved survival by standardizing care and tailoring treatment intensity to age and tumor type.
Despite evolving definitions, the arc of progress is clear: from hidden cause to visible target, from symptom relief alone to combined approaches aimed at cure while protecting brain development. Today, the history of Choroid plexus cancer informs practical steps—prompt imaging for early symptoms of Choroid plexus cancer, timely referral to specialized centers, and discussions about genetic testing—so families and clinicians can act sooner and with greater confidence.