Papilloma of the choroid plexus is a rare benign type of choroid plexus tumor, accounting for 1% of all brain tumors, often occurring in the fourth ventricle (in adults) and the lateral ventricle (in ...
Comprehensive, easy-to-understand information about this condition
How we create this content →Documentation on choroid plexus papilloma is limited due to its rarity and the fact that it affects fewer than 1 in 100,000 people. This extreme rarity leads to challenges in conducting systematic clinical studies and gathering comprehensive data. Additionally, the genetic basis of this condition has not been fully characterized, which contributes to the gaps in understanding its pathophysiology.
The clinical features of choroid plexus papilloma include hydrocephalus (80-99% of cases), abnormal nervous system morphology (80-99%), and the presence of the tumor itself (80-99%). Other symptoms may include hypertonia, cognitive impairment, seizures, and visual impairment, each occurring in 5-29% of cases. These symptoms are often related to the effects of increased intracranial pressure and the tumor's location.
To navigate your care effectively, consider consulting with a neuro-oncologist or a neurosurgeon who specializes in brain tumors, particularly those with experience in pediatric cases if applicable. You may also benefit from genetic counseling, especially given the autosomal dominant inheritance pattern. For resources, you can explore the Genetic and Rare Diseases Information Center at rarediseases.info.nih.gov for more information and support.
Currently, there are no orphan drug designations for choroid plexus papilloma, but there is one active clinical trial. This trial may provide opportunities for patients to participate in research that could lead to new insights or treatments. For more information on this trial, please visit the ClinicalTrials.gov website at https://clinicaltrials.gov/search?cond=choroid%20plexus%20papilloma.
Actionable guidance for navigating care for choroid plexus papilloma
To navigate your care effectively, consider consulting with a neuro-oncologist or a neurosurgeon who specializes in brain tumors, particularly those with experience in pediatric cases if applicable. You may also benefit from genetic counseling, especially given the autosomal dominant inheritance pattern. For resources, you can explore the Genetic and Rare Diseases Information Center at rarediseases.info.nih.gov for more information and support.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Documentation on choroid plexus papilloma is limited due to its rarity and the fact that it affects fewer than 1 in 100,000 people. This extreme rarity leads to challenges in conducting systematic clinical studies and gathering comprehensive data. Additionally, the genetic basis of this condition has not been fully characterized, which contributes to the gaps in understanding its pathophysiology.
The clinical features of choroid plexus papilloma include hydrocephalus (80-99% of cases), abnormal nervous system morphology (80-99%), and the presence of the tumor itself (80-99%). Other symptoms may include hypertonia, cognitive impairment, seizures, and visual impairment, each occurring in 5-29% of cases. These symptoms are often related to the effects of increased intracranial pressure and the tumor's location.
To navigate your care effectively, consider consulting with a neuro-oncologist or a neurosurgeon who specializes in brain tumors, particularly those with experience in pediatric cases if applicable. You may also benefit from genetic counseling, especially given the autosomal dominant inheritance pattern. For resources, you can explore the Genetic and Rare Diseases Information Center at rarediseases.info.nih.gov for more information and support.
Currently, there are no orphan drug designations for choroid plexus papilloma, but there is one active clinical trial. This trial may provide opportunities for patients to participate in research that could lead to new insights or treatments. For more information on this trial, please visit the ClinicalTrials.gov website at https://clinicaltrials.gov/search?cond=choroid%20plexus%20papilloma.
Actionable guidance for navigating care for choroid plexus papilloma
To navigate your care effectively, consider consulting with a neuro-oncologist or a neurosurgeon who specializes in brain tumors, particularly those with experience in pediatric cases if applicable. You may also benefit from genetic counseling, especially given the autosomal dominant inheritance pattern. For resources, you can explore the Genetic and Rare Diseases Information Center at rarediseases.info.nih.gov for more information and support.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Documentation on choroid plexus papilloma is limited due to its rarity and the fact that it affects fewer than 1 in 100,000 people. This extreme rarity leads to challenges in conducting systematic clinical studies and gathering comprehensive data. Additionally, the genetic basis of this condition has not been fully characterized, which contributes to the gaps in understanding its pathophysiology.
The clinical features of choroid plexus papilloma include hydrocephalus (80-99% of cases), abnormal nervous system morphology (80-99%), and the presence of the tumor itself (80-99%). Other symptoms may include hypertonia, cognitive impairment, seizures, and visual impairment, each occurring in 5-29% of cases. These symptoms are often related to the effects of increased intracranial pressure and the tumor's location.
To navigate your care effectively, consider consulting with a neuro-oncologist or a neurosurgeon who specializes in brain tumors, particularly those with experience in pediatric cases if applicable. You may also benefit from genetic counseling, especially given the autosomal dominant inheritance pattern. For resources, you can explore the Genetic and Rare Diseases Information Center at rarediseases.info.nih.gov for more information and support.
Currently, there are no orphan drug designations for choroid plexus papilloma, but there is one active clinical trial. This trial may provide opportunities for patients to participate in research that could lead to new insights or treatments. For more information on this trial, please visit the ClinicalTrials.gov website at https://clinicaltrials.gov/search?cond=choroid%20plexus%20papilloma.
Actionable guidance for navigating care for choroid plexus papilloma
To navigate your care effectively, consider consulting with a neuro-oncologist or a neurosurgeon who specializes in brain tumors, particularly those with experience in pediatric cases if applicable. You may also benefit from genetic counseling, especially given the autosomal dominant inheritance pattern. For resources, you can explore the Genetic and Rare Diseases Information Center at rarediseases.info.nih.gov for more information and support.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Inheritance patterns describe how genetic conditions are passed from parents to children.
Research studies investigating treatments and therapies for this condition.
Active Trials
Total Trials
Data from ClinicalTrials.gov Jan 8, 2026
Consider asking your healthcare providers these condition-specific questions
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
AI-Generated Content: This summary was generated using AI. Content has been fact-checked. Always consult with qualified healthcare providers for medical guidance.
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