Choroid plexus tumor: symptoms, treatment, and hopeful outlook

If you've just heard the words "choroid plexus tumor," take a slow breath with me. It's a rare diagnosis, and it's completely normal to feel a swirl of worry and questions. Here's the good news right up front: most choroid plexus tumors are benign (called choroid plexus papillomas), and surgery often cures them. Still, even benign brain tumors can cause real symptoms by increasing pressure in the brainthink headaches, nausea, vision changesso getting thoughtful, timely care matters.

There's also a smaller group called choroid plexus carcinoma. These are cancerous, grow faster, and can spread within the brain and spinal fluid. They typically need a combination of surgery plus chemotherapy and/or radiation. The most important thing? Early, expert care makes a real difference. You deserve a trusted team, straight answers, and a plan you can understand. Let's walk through this together, step by step.

What is it?

A choroid plexus tumor starts in the choroid plexusthe delicate, frond-like tissue inside the brain's fluid-filled spaces (the ventricles). This tissue makes cerebrospinal fluid (CSF), which cushions your brain and spinal cord. When a tumor grows here, it can disrupt the normal flow or production of CSF, leading to hydrocephalus (a buildup of fluid) and increased pressure inside the skull.

Where these start and who gets them

The choroid plexus sits inside the ventricles (picture tiny coral reefs waving gently inside a sea of CSF). Tumors can grow in the lateral ventricles, third ventricle, or fourth ventricle. In children, especially infants and toddlers, these tumors more often appear in the lateral ventricles. In adults, they're seen more often in the fourth ventricle near the back of the brain. While choroid plexus tumors are rare overall, they're relatively more common in very young childrenbut they can occur at any age.

Choroid plexus basics

The choroid plexus produces CSF. That fluid flows through the ventricles and around the brain and spinal cord, delivering nutrients and clearing waste. A tumor can cause problems by overproducing CSF or blocking its normal floweither way, pressure rises. That's why symptoms often look like "pressure symptoms."

Age patterns

Most cases occur in infants and young children, but adults can absolutely be affected. The type and location can differ with age, which helps doctors narrow the diagnosis during imaging.

Types and grades

Not all choroid plexus tumors behave the same. Pathologists (the doctors who analyze tissue under the microscope) categorize them by grade:

Grade 1: Choroid plexus papilloma

Benign and typically slow-growing. With complete surgical removal, many people do very well long term.

Grade 2: Atypical choroid plexus papilloma

Still considered noncancerous, but with features that suggest a higher risk of coming back. These may need closer follow-up and, sometimes, additional treatment if not fully removable or if they recur.

Grade 3: Choroid plexus carcinoma

Malignant. These grow faster and have the potential to spread through the CSF pathways. Treatment usually includes surgery plus chemotherapy and/or radiation.

MRI "look" and location patterns

On MRI, these tumors often appear vividly with contrast because they're richly vascular (lots of blood supply). In children, lateral ventricles are common sites; in adults, the fourth ventricle is a frequent location. According to expert centers such as the National Cancer Institute and Cedars-Sinai, this typical "look and location" helps guide initial planning, but pathology still provides the final answer.

Key symptoms

What does a choroid plexus tumor feel like? It depends on the person's age, tumor size, and whether CSF flow is blocked. Think of the skull like a closed box; if fluid builds up, pressure rises. That pressure drives many of the classic brain tumor symptoms you may see discussed under "brain tumor symptoms."

Signs of increased pressure

Watch for headaches that are often worse in the morning, nausea or vomiting (especially on waking), sleepiness, blurred or double vision, dizziness, balance problems, and sometimes seizures. Not every person will have all of these. Some people just notice a steady shiftmore fatigue, more headaches, a sense that vision isn't quite right.

Infants and toddlers

In very young children, the signs can look different. Because their skull bones haven't fully fused yet, the head may enlarge. You might see a bulging soft spot (fontanelle), irritability that's hard to soothe, feeding challenges, poor weight gain, or delayed developmental milestones. If your parent radar is pinging, trust itbring concerns to your pediatrician promptly.

When to seek urgent care

If there are persistent morning headaches with vomiting, rapidly worsening vision, significant sleepiness, or a new seizure, it's time to seek urgent or emergency care. You know your body or your child bestif something feels seriously off, don't wait.

Causes

It's natural to ask "Why did this happen?" In most cases, choroid plexus tumors occur sporadicallythere's no obvious cause. The symptoms are often driven by CSF overproduction or blockage, leading to hydrocephalus. Please remember: nothing you did caused this, and nothing you could have done would have guaranteed prevention.

Genetic links

Some malignant cases, especially choroid plexus carcinoma, can be tied to mutations in TP53, a gene that helps regulate cell growth. There's an association with Li-Fraumeni syndrome, an inherited condition that increases the risk of certain cancers. In those scenarios, doctors may recommend genetic counseling and testing. According to leading centers such as the National Cancer Institute and Dana-Farber Cancer Institute, genetic testing is considered when carcinoma is diagnosed or there's a strong family history of cancers at young ages. You and your care team can decide together if this applies to you.

Diagnosis

Getting to the bottom of things usually starts with imaging and a careful neurological exam. The team is often multidisciplinaryyou'll feel like you've met a small army, but each specialist has a role.

The care team

You may meet a neurologist (symptoms and exam), a neurosurgeon (surgery and pressure management), and a neuro-oncologist (treatment planning, if cancer is suspected). A neuropathologist will analyze tissue from a biopsy or the surgery to confirm the exact type and grade. According to guidance shared by organizations such as the National Cancer Institute, working with a center that treats choroid plexus tumors regularly can streamline all of this.

Tests and imaging

MRI with contrast is the go-to testit provides rich detail and helps the team understand size, location, and involvement with nearby structures. Sometimes a CT scan is used, especially in emergency situations. In select pediatric cases, a lumbar puncture (spinal tap) may be considered to check the CSF for tumor cells, particularly if carcinoma is suspected. Ultimately, a definitive diagnosis comes from looking at the tumor tissue under the microscope, either from a biopsy or the main surgery.

Staging and spread

Choroid plexus carcinoma can spread along CSF pathways, so doctors may order MRI scans of the spine to look for "drop metastases." This is uncommon in papillomas. If carcinoma is confirmed, your team will map any spread to shape the treatment plan.

Treatment options

Here's where we focus on action. The plan depends on the type and grade of the tumor, your age, your overall health, and whether there's any spread. Your team should explain the reasoning behind each step so you feel informed and empowered.

First-line: surgery

Surgery is usually step one. Goals include relieving pressure, obtaining tissue for diagnosis, and removing as much tumor as safely possible (what surgeons call "gross total resection"). For choroid plexus papilloma, complete removal often leads to cure. After surgery, many people notice headaches and nausea improving as the pressure normalizes. It's common to feel tired; the brain has been through a lot, and rest is therapeutic.

Outcomes after surgery

When a papilloma is fully removed, long-term outcomes are generally excellent. If the tumor can't be completely removed because it's too risky, you and your surgeon may discuss surveillance (watchful waiting) or additional therapy. For atypical papilloma, the team may monitor more closely and consider tailored follow-up plans to catch recurrence early.

When additional therapy is needed

For choroid plexus carcinomaand sometimes for atypical or recurrent tumorsthere may be more steps after surgery.

Radiation therapy

Radiation can help control or eliminate residual tumor. In children, specialists balance the benefits of radiation with the potential long-term effects on developing brains. Techniques and doses are carefully chosen to minimize those effects. The decision to use radiation is highly individualized.

Chemotherapy

Chemotherapy may be used to shrink the tumor, treat spread in the CSF, or reduce the need for higher-dose radiation. Some regimens are given intravenously; in select cases, chemotherapy can be delivered into the CSF (intrathecal) to target spread along the spinal fluid. According to pediatric neuro-oncology programs such as Dana-Farber, the exact drugs and timing depend on age, tumor behavior, and response to surgery.

Clinical trials

Because choroid plexus tumors are rare, clinical trials are vital for improving care. Trials may explore targeted therapy, immunotherapy, or supportive care approaches. If you're curious about eligibility, ask your team; according to the National Cancer Institute's resources on rare pediatric brain tumors, new options may be available through specialized centers. You might see language like "phase II trial" or "targeted therapy study"it's all part of moving the field forward.

Managing hydrocephalus and symptoms

Hydrocephalus can be managed in several ways. In emergencies, surgeons may place an external ventricular drain to relieve pressure quickly. Some patients need a shunt (a small tube that diverts CSF to the abdomen) or an endoscopic procedure to improve CSF flow. Steroids may reduce swelling around the tumor, and antiseizure medications are used if seizures occur. These supportive steps can make a big difference in how you feel, even while the bigger treatment plan unfolds.

Prognosis

Let's talk about outlook in plain language. Prognosis depends on tumor type and grade, how much the surgeon can safely remove, whether there's spread, specific genetic changes (like TP53), age, and overall health. For many people with choroid plexus papilloma, especially after complete resection, the outlook is very encouraging. Atypical papilloma carries a higher risk of recurrence, so close follow-up is key. Carcinoma typically needs a full-court presssurgery plus chemotherapy and/or radiationfollowed by vigilant surveillance.

Survival and recurrence

There's no one-size-fits-all number, and it's okay to ask your doctors for context specific to you or your child. What matters most is that treatment decisions align with the tumor's behavior and your goals. For benign brain tumor types like papilloma, cure rates after total removal are high. Carcinoma is more complex but absolutely treatable, especially with a team experienced in choroid plexus cancer.

Follow-up care

Expect MRI scans on a regular schedulemore often at first, then spaced out over time if all is stable. Children who receive chemotherapy or radiation may also have neurocognitive testing and endocrine (hormone) evaluations to support learning, growth, and development. Physical, occupational, and speech therapy can be part of the plan. If school re-entry is in the picture, ask about individualized education plans or 504 accommodations; hospitals with pediatric neuro-oncology programs often provide education liaisons who coordinate with schools.

Daily life tips

Let's bring it down to the day-to-day. Appointments, scans, and big decisions can feel like spinning plates. It helps to write things downsymptoms, questions, medications, how you're sleeping, what triggers headaches. Bring someone with you to appointments to take notes or just be your second set of ears.

Preparing for appointments and surgery

Ask your surgeon what "success" looks like in your case. Will they aim for a gross total resection? What are the risks, and how are they managed? How long might you be in the hospital, and what's the plan for pain control and nausea? It's perfectly okay to seek a second opinion. According to resources from major centers like Cedars-Sinai and the National Cancer Institute, second opinions can clarify options and give you peace of mindespecially with rare tumors.

Side effects and recovery

In the short term, expect fatigueit's common after brain surgery. Headaches can linger but usually improve as swelling settles. Follow incision-care instructions to the letter. Over the longer term, some people experience changes in concentration, memory, or balance. Children, whose brains are still developing, may be more sensitive to treatment effects. That's why pediatric care teams monitor learning, behavior, and hormone function. Healing isn't just physicalit's cognitive and emotional, too.

Support and mental health

There's a lot to carry here. You don't need to carry it alone. Hospital social workers can connect you with financial resources, transportation support, and therapy services. Neuropsychologists can help you understand and address cognitive changes. Survivorship clinics coordinate long-term follow-up and help you plan for the future. Caregivers, please remember: your rest matters too. Even a 10-minute walk outside can reset your nervous system.

It may also help to learn from others' experiences. Patient communities can be a lifeline when you want practical tips from people who've been there. If you're exploring treatment centers or research updates, you might find it helpful to review reputable sources such as the National Cancer Institute's pages on rare CNS tumors or pediatric neuro-oncology overviews from Dana-Farber; both provide patient-friendly explanations and reflect current standards of care.

Know the difference

The wording can be confusing, so let's clear it up. "Choroid plexus tumor" is the umbrella term that includes both benign and malignant types. "Choroid plexus cancer" generally refers to choroid plexus carcinomathe malignant form. Even a benign brain tumor can cause serious symptoms because of pressure and location, while malignant tumors add the risk of faster growth and spread. The language matters because it guides treatment decisions. If you're unsure how your team is using these terms, ask them to clarify. You deserve crystal-clear answers.

Related conditions

Choroid plexus papilloma vs choroid plexus carcinoma: both start in the same tissue, but they behave differently. Papillomas are benign and often cured with surgery. Carcinomas are malignant; they usually require surgery plus chemotherapy and/or radiation and closer follow-up. Different names, different strategiessame goal: the best possible outcome for you.

A quick story

I once spoke with a parent whose toddler had a choroid plexus papilloma. The signs were so subtle at firstjust crankiness and morning vomiting. They felt silly walking into the pediatrician's office yet again. But their persistence led to an MRI, then surgery. The tumor was removed completely, and within weeks, their little one's belly laughs were back. Not every story is that straightforward, of course. But it's a reminder that you know yourself or your child bestand that persistence can change everything.

Your next steps

If you suspect a choroid plexus tumor or have a new diagnosis, here's a simple way to move forward with confidence:

1) Get imaging with MRI if not already done. 2) Meet with a neurosurgeon who has experience with intraventricular tumors. 3) Ask about the plan for pressure relief and tissue diagnosis. 4) Clarify whether the team suspects papilloma, atypical papilloma, or carcinomaand what that means for treatment. 5) If carcinoma is on the table, ask about chemotherapy, radiation, and clinical trials. 6) Consider a second opinion at a major center, especially for pediatric cases. You are not "difficult" for asking questionsyou are being a great advocate.

Encouragement

It's okay to be scared. It's okay to have a thousand questions. And it's absolutely okay to lean on othersmedical teams, friends, online communitieswhile you process and plan. Remember: most choroid plexus tumors are benign, and surgery often brings relief and cure. For cancerous tumors, treatment can be demanding, but there's a path forward, guided by specialists who do this every day. You are not alone in this.

Conclusion

Choroid plexus tumors are rare, and rare can feel isolating. But you're already doing the brave thinglearning, asking, and planning. Most cases are benign choroid plexus papillomas, and many are cured with surgery. When the tumor is malignantchoroid plexus carcinomathe approach is more aggressive and team-based, often combining surgery with chemotherapy and/or radiation. The most valuable step you can take is to connect with an experienced center and a team you trust. Ask your questions. Take notes. Bring support. And keep in mind: outcomes are often better than our fears suggest. What questions are still on your mind? If you want help understanding a scan result, a pathology term, or the pros and cons of a treatment option, ask awayI'm here to help you make sense of it.

Disclaimer: This article is for informational purposes only and does not constitute medical advice. Always consult with a healthcare professional before starting any new treatment regimen.