ConditionBrain & nerves
Choroid Plexus Papilloma (CPP)
A choroid plexus papilloma (CPP) is a rare, slow-growing, noncancerous brain tumor that arises from the choroid plexus, the tissue inside the brain's fluid-filled ventricles that makes cerebrospinal fluid (CSF). It is most common in babies and children under 2, though adults can have it too. It usually causes hydrocephalus, a buildup of fluid that raises pressure in the skull. Complete surgical removal is usually curative, and the long-term outlook is excellent.
Also called: CPP, choroid plexus tumor (benign type), WHO grade 1 choroid plexus tumor
At a glance
- What it is
- A benign (WHO grade 1) tumor of the CSF-producing tissue in the brain's ventricles.
- Who gets it
- Mostly children under 2, especially infants. Also adults, usually in the fourth ventricle.
- Main effect
- Hydrocephalus from extra CSF production and blocked fluid flow.
- Diagnosis
- Ultrasound in babies, MRI with contrast, confirmed by tissue examination.
- Treatment and outlook
- Complete surgical removal, with excellent long-term survival.
When to get help
Call 911 or go to the ER
- A baby with a bulging soft spot, vomiting, extreme sleepiness or irritability, or eyes that look downward (sunsetting eyes)
- Severe headache with vomiting, confusion, drowsiness or a seizure
- A child or adult with a shunt who develops headache, vomiting or drowsiness (possible shunt blockage)
See a doctor within a day or two
- A baby's head growing faster than expected on growth charts
- Morning headaches with vomiting in a child, getting worse over days to weeks
- New double vision, unsteadiness or loss of developmental skills
Book a routine appointment
- Missed head circumference measurements in a baby's checkups
- Known CPP after surgery: keep scheduled MRI follow-up
What the choroid plexus does
The brain has four interconnected fluid spaces called ventricles. Inside them is the choroid plexus, a frond-like tissue that produces cerebrospinal fluid, which cushions the brain and spinal cord and is constantly made and reabsorbed. A choroid plexus papilloma is an overgrowth of this tissue that looks like a cauliflower.
Types of choroid plexus tumors
| Type | WHO grade | Behavior |
|---|---|---|
| Choroid plexus papilloma | 1 | Benign, slow-growing, rarely recurs after full removal |
| Atypical choroid plexus papilloma | 2 | More cell division, higher chance of recurrence |
| Choroid plexus carcinoma | 3 | Malignant, can spread through CSF. Strongly linked to Li-Fraumeni syndrome |
Where they occur
- Children: most often in the lateral ventricles, the large fluid spaces in each cerebral hemisphere.
- Adults: most often in the fourth ventricle, at the back of the brain near the cerebellum and brainstem.
Symptoms
Most symptoms come from hydrocephalus, caused by the tumor making extra CSF and by blocking its flow.
In babies
- Head growing faster than normal
- A bulging or tense soft spot (fontanelle) and widened skull seams
- Vomiting and poor feeding
- Irritability or unusual sleepiness
- Eyes that seem to look down ("sunsetting")
- Delayed development or loss of skills
In older children and adults
- Headaches, often worse in the morning or when lying down
- Nausea and vomiting
- Blurred or double vision, swelling of the optic nerves (papilledema)
- Unsteadiness and poor coordination (fourth ventricle tumors)
- Seizures, uncommonly
- Drowsiness and personality changes
Causes
The cause of most CPPs is unknown. They are not caused by anything parents did during pregnancy. Some occur with genetic conditions such as Aicardi syndrome, and choroid plexus carcinomas are associated with inherited TP53 mutations (Li-Fraumeni syndrome). Genetic counseling may be offered, especially for carcinomas.
Diagnosis
- Head circumference monitoring at well-baby visits can flag rapid head growth.
- Cranial ultrasound through the open soft spot in babies can show hydrocephalus and a mass.
- MRI with contrast: the main test. CPPs appear as lobulated, brightly enhancing masses inside a ventricle. MRI of the spine may also be done to check for spread, mainly when carcinoma is suspected.
- CT scan in emergencies.
- Pathology: the diagnosis and grade are confirmed when the removed tumor is examined under the microscope.
Treatment
Surgery
Complete surgical removal (gross total resection) is the treatment of choice and is usually curative for CPP. These tumors have a rich blood supply, so surgery can involve significant bleeding, especially in small babies. Surgeons sometimes block the tumor's blood vessels beforehand (embolization) or remove it in stages.
Managing hydrocephalus
Removing the tumor often resolves hydrocephalus because CSF overproduction stops. Some children need a temporary drain or a permanent ventriculoperitoneal (VP) shunt, a tube that drains fluid from the brain to the belly. Shunts can block or become infected, so families learn the warning signs of shunt malfunction.
Additional treatment
For a CPP that is completely removed, no further treatment is usually needed. Chemotherapy or radiation may be considered for incompletely removed atypical papillomas, recurrence or choroid plexus carcinoma.
Outlook
The outlook for completely removed choroid plexus papilloma is excellent, with long-term survival close to 100 percent. Recurrence is uncommon after complete removal. Children who had significant hydrocephalus before treatment may have developmental effects and benefit from early intervention services. Follow-up with periodic MRI scans checks for recurrence.
Frequently asked questions
Is a choroid plexus papilloma cancer?
No. A choroid plexus papilloma is a benign (WHO grade 1) tumor that grows slowly and does not invade the brain or spread. It can still cause serious problems by raising pressure in the skull, so it needs treatment. Its malignant counterpart is called choroid plexus carcinoma.
What are the symptoms of choroid plexus papilloma in babies?
Babies typically show signs of fluid buildup: a rapidly growing head, a bulging soft spot, vomiting, poor feeding, irritability or sleepiness, and eyes that look downward. These signs need urgent medical assessment.
How is choroid plexus papilloma treated?
The main treatment is surgery to remove the whole tumor, which is usually curative. Hydrocephalus often improves after removal, but some children need a temporary drain or a permanent shunt. Further treatment is rarely needed unless the tumor is atypical, incompletely removed or recurs.
What is the prognosis for choroid plexus papilloma?
The prognosis is excellent. When the tumor is completely removed, long-term survival is close to 100 percent and recurrence is uncommon. Some children have lasting effects from hydrocephalus and benefit from developmental support. Regular follow-up MRI scans are recommended.
Sources
This guide was written from current patient and clinical guidance, including:
- National Cancer Institute: Choroid plexus tumors
- American Association of Neurological Surgeons: Hydrocephalus; Brain tumors
- WHO Classification of Tumours of the Central Nervous System (5th edition)
- National Organization for Rare Disorders (NORD): Choroid plexus tumors
- St. Jude Children's Research Hospital: Choroid plexus tumors