National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Choroid plexus papilloma


Other Names:
Papilloma of choroid plexus
Categories:
Choroid plexus papilloma (CPP) ia a non-cancerous (benign) tumor of the choroid plexus, a network of blood vessels in the brain which surrounds the ventricles and produces the fluid surrounding the brain and spinal cord (cerebrospinal fluid, or CSF).[1] CPPs most commonly occur in children but may occur in adults.[2] Symptoms are generally due to increased secretion of CSF by tumor cells, causing hydrocephalus and subsequent intracranial pressure.[2] Affected individuals may experience headaches, nausea and vomiting, drowsiness, ocular or gaze palsies, optic nerve swelling (papilledema), visual disturbances, and possible blindness. Infants, especially those with a tumor in the third ventricle, can present with hydrocephalus or macrocephaly.[2] Some cases of CPP may result from a mutation in the TP53 gene.[3] Complete tumor removal often cures the condition and may also relieve the hydrocephalus.[1]
Last updated: 12/10/2012

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Choroid plexus papilloma 0200022
Hydrocephalus
Too much cerebrospinal fluid in the brain
0000238
5%-29% of people have these symptoms
Cognitive impairment
Abnormality of cognition
Cognitive abnormality
Cognitive defects
Cognitive deficits
Intellectual impairment
Mental impairment
[ more ] 		0100543
Hemiplegia/hemiparesis
Paralysis or weakness of one side of body
0004374
Hypertonia 0001276
Seizure 0001250
Visual impairment
Impaired vision
Loss of eyesight
Poor vision
[ more ] 		0000505
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance 0000006
Autosomal recessive inheritance 0000007
Headache
Headaches
0002315
Nausea 0002018
Papilledema 0001085
Vomiting
Throwing up
0002013
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Last updated: 7/1/2020

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

  • The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.
The prognosis for choroid plexus papilloma (CPP) is determined mainly by how completely the lesion is removed during surgery. Complete removal of intraventricular CPPs almost always cures the condition and it has been estimated that the survival rate at 5 years for CPPs after removal is approximately 100%. Examples of rare complications have been described and generally involve neurologic deficits from the surgical procedure.[2]

Although there may not be residual effects of the CPP specifically, the extent and duration of the presence of hydrocephaly can affect cognitive and/or physical development in affected individuals.[4] Hydrocephalus may continue after surgery in some cases and is then often managed by CSF shunting. Complications that result in neurological or psychological problems may influence overall outcome.[2]
Last updated: 12/11/2012

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Choroid plexus papilloma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Choroid plexus papilloma. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I have a close friend whose neonate was born with severe hydrocephalus which was diagnosed at 32 weeks gestation. At 3 weeks of age the baby was diagnosed with a choroid plexus papilloma through MRI. The physicians plan to do a spinal MRI to see if it has spread. Have you found that children developing this condition in utero are more or less likely to have long term complications? See answer


  1. Choroid Plexus. American Brain Tumor Association. 2012; http://www.abta.org/understanding-brain-tumors/types-of-tumors/choroid-plexus.html. Accessed 12/10/2012.
  2. Cheryl Ann Palmer. Choroid Plexus Papilloma. Medscape Reference. March 8, 2012; http://emedicine.medscape.com/article/250795-overview. Accessed 12/10/2012.
  3. Papilloma of Choroid Plexus. OMIM. August 21, 2002; http://omim.org/entry/260500. Accessed 12/10/2012.
  4. Hydrocephalus Fact Sheet. NINDS. July 27, 2015; http://www.ninds.nih.gov/disorders/hydrocephalus/detail_hydrocephalus.htm. Accessed 12/16/2015.