Choroid Plexus Papilloma: Symptoms, Diagnosis and Treatment

Written by Medicover Team and Medically Reviewed by Dr Prachi Rahul Pawar , Neurologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Choroid plexus papilloma (CPP) is a rare, usually benign brain tumor that arises from the choroid plexus, the tissue responsible for producing cerebrospinal fluid (CSF). It most commonly affects infants and young children but can also occur in adults. The tumor may increase cerebrospinal fluid production or block its normal flow, leading to hydrocephalus and increased pressure inside the skull.

Common symptoms include headaches, nausea, vomiting, balance problems, irritability, and developmental delays in children. Diagnosis is confirmed through neurological examination, MRI or CT scans, and histopathological evaluation after surgical removal, with surgery being the primary treatment and an excellent prognosis in most cases.

What are the Causes of Choroid Plexus Papilloma?

The exact aetiology of choroid plexus papilloma remains unclear. However, genetic predisposition may play a role, with associations reported in certain hereditary cancer syndromes. Further research is ongoing to better understand the molecular mechanisms involved in the development of CPP.

Causes of Choroid Plexus Papilloma

Genetic Mutations: Mutations in genes that regulate cell growth and division may contribute to the abnormal proliferation of choroid plexus cells.
Developmental Factors: Since CPP commonly occurs in infants and young children, abnormalities during early brain development may contribute to its formation.
Environmental Factors: Although uncommon, exposure to environmental toxins or radiation may increase the risk of developing CPP.

What are the Symptoms of Choroid Plexus Papilloma?

The clinical presentation of choroid plexus papilloma depends on the tumor size, location, and the degree of cerebrospinal fluid (CSF) obstruction. Common symptoms include:

Hydrocephalus: Caused by obstruction of cerebrospinal fluid flow, leading to increased intracranial pressure.
Headaches: Often severe and persistent because of increased pressure inside the skull.
Nausea and Vomiting: Frequently associated with elevated intracranial pressure.
Visual Disturbances: Caused by increased pressure affecting the optic pathways.
Gait Instability and Coordination Problems: Occur when tumors involve the fourth ventricle or nearby cerebellar structures.

Common Symptoms of Choroid Plexus Papilloma in Adults

Headaches
Nausea and Vomiting
Vision Problems
Balance and Coordination Problems
Cognitive Changes
Seizures

When to See a Doctor for Choroid Plexus Papilloma?

If you or your child develops symptoms suggestive of choroid plexus papilloma, consult a Neurologist promptly. Early diagnosis and treatment can help relieve increased pressure inside the skull, prevent neurological complications, and improve long-term outcomes.

If you experience persistent or severe headaches that worsen over time.
If you develop nausea or vomiting, especially when associated with headaches.
If you notice blurred vision, double vision, or other unexplained vision changes.
If you experience balance problems, difficulty walking, or poor coordination.
If you develop seizures or unexplained episodes of loss of consciousness.
If your child has an enlarged head, irritability, developmental delays, or feeding difficulties.
If brain imaging reveals a tumor or hydrocephalus requiring further evaluation.
If your symptoms persist or worsen, consult a Neurologist promptly for a comprehensive neurological evaluation, brain imaging, diagnosis, and appropriate treatment.

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How is Choroid Plexus Papilloma Diagnosed?

Radiological Assessment

Radiological imaging plays a key role in diagnosing choroid plexus papilloma. Magnetic resonance imaging (MRI) is the preferred imaging technique because it provides detailed information about the tumor's size, location, and its effects on surrounding brain structures.

CPP typically appears as a well-circumscribed, lobulated mass with vivid contrast enhancement on MRI scans. A CT scan may also be performed when MRI is not suitable and can identify calcifications within the tumor in some patients.

Differential Diagnosis

The differential diagnosis includes other intraventricular tumors such as ependymomas, medulloblastomas, and central neurocytomas. Correctly distinguishing CPP from these conditions is important because treatment strategies and prognosis differ significantly.

What are the Treatment Options for Choroid Plexus Papilloma?

The primary goal of choroid plexus papilloma treatment is to completely remove the tumor, relieve symptoms, and prevent complications such as hydrocephalus caused by excessive cerebrospinal fluid (CSF) production.

Surgical Resection

The primary treatment for choroid plexus papilloma is surgery. Complete removal of the tumor often resolves symptoms and hydrocephalus because of the tumor's benign nature. The surgical approach depends on the tumor's location and aims to achieve complete removal while minimizing neurological damage.

Adjuvant Therapies

If complete surgical removal is not possible or if the tumor has atypical and aggressive features, additional treatments such as radiation therapy may be recommended. Chemotherapy is rarely required because choroid plexus papilloma is generally a low-grade tumor.

Postoperative Care and Monitoring

Postoperative care includes monitoring for complications such as infection, bleeding, and persistent hydrocephalus. Long-term follow-up with regular imaging studies is essential to detect recurrence, particularly after subtotal tumor removal.

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What are the Complications of Choroid Plexus Papilloma?

Choroid plexus papilloma (CPP) is a rare benign brain tumor, but if left untreated, it can cause several complications because of increased cerebrospinal fluid production and elevated pressure within the skull.

Common Complications of Choroid Plexus Papilloma

Hydrocephalus: Excess cerebrospinal fluid production or blockage of CSF flow can cause hydrocephalus, leading to headaches, nausea, vomiting, and potential brain damage if untreated.
Increased Intracranial Pressure: Tumor growth can increase pressure within the skull, causing headaches, nausea, vomiting, vision problems, and drowsiness.
Seizures: Irritation of nearby brain tissue may trigger seizures in some individuals.
Neurological Deficits: Persistent tumor growth or surgical complications may result in long-term motor, sensory, or cognitive impairments.

Managing Complications

Early diagnosis and timely surgical treatment are essential to reduce the risk of complications. After surgery, care focuses on maintaining normal cerebrospinal fluid circulation, preventing infections, monitoring neurological function, and detecting any recurrence through regular follow-up imaging.

What is the Prognosis of Choroid Plexus Papilloma?

The prognosis for patients with choroid plexus papilloma is generally excellent, especially when the tumor is completely removed through surgery. The five-year survival rate exceeds 90% in most cases. Patients with atypical choroid plexus papilloma may require closer follow-up and, in some situations, additional treatments such as radiation therapy.

Frequently Asked Questions

1. What are the symptoms of Choroid Plexus Papilloma?

Choroid Plexus Papilloma Symptoms may include headaches, nausea, and developmental delays in children, indicating a tumor in the choroid plexus of the brain.

2. What causes Choroid Plexus Papilloma?

Choroid Plexus Papilloma Causes are often unclear but may involve genetic predisposition affecting the growth of the choroid plexus cells.

3. How is Choroid Plexus Papilloma diagnosed?

Choroid Plexus Papilloma Diagnosis typically involves imaging studies such as MRI or CT scans and biopsy to confirm the tumor type.

4. What are the treatment options for Choroid Plexus Papilloma?

Choroid Plexus Papilloma Treatment often involves surgical resection, with monitoring for recurrence, especially in pediatric cases.

5. What is the prognosis for Choroid Plexus Papilloma?

The prognosis of Choroid Plexus Papilloma is generally favorable, particularly with complete surgical removal of the tumor, but follow-up care is essential.