Choroid Plexus Papilloma
Choroid plexus papilloma (CPP) is a rare, usually benign brain tumor that arises from the tissue in the ventricles of the brain called the choroid plexus. This tissue is responsible for producing cerebrospinal fluid (CSF), the clear liquid that surrounds and protects the brain and spinal cord. While choroid plexus papillomas are not cancerous, their location and effect on CSF production and flow can cause significant neurological problems if left untreated.
Understanding the Condition
The choroid plexus is located within the brain’s ventricular system. Its primary role is to generate CSF, which cushions the brain, removes waste products, and helps maintain stable pressure within the skull. When a papilloma develops in this tissue, it forms a small, finger-like growth that resembles healthy tissue but produces excess fluid and can block the normal flow of CSF.
Because of this, one of the hallmark complications of CPP is hydrocephalus—a buildup of fluid within the brain’s ventricles. This can lead to increased intracranial pressure and a variety of symptoms depending on the patient’s age and the tumor’s location.
Who Is Affected?
Choroid plexus papillomas are most commonly diagnosed in children, particularly under the age of two, though they can occur at any age. In infants and young children, they often appear in the lateral ventricles, while in adults, they are more likely to form in the fourth ventricle at the back of the brain. CPP accounts for less than 1% of all brain tumors but up to 4% of brain tumors in children.
Symptoms
Symptoms vary based on the tumor’s size, location, and how much it disrupts CSF flow. Common signs include:
- Headaches (often worse in the morning due to fluid buildup)
- Nausea and vomiting
- Enlarged head size in infants or bulging of the soft spot (fontanelle)
- Balance problems or difficulty walking
- Seizures
- Changes in vision or double vision
In young children, parents may also notice irritability, developmental delays, or poor feeding.
Diagnosis
Doctors rely on imaging studies to identify CPP. Magnetic resonance imaging (MRI) provides detailed pictures of the brain’s ventricles and can often distinguish papillomas from other types of tumors. Computed tomography (CT) scans may also be used to assess ventricular size and fluid buildup. In some cases, additional tests like cerebrospinal fluid sampling may be ordered to rule out infection or other conditions.
Treatment Options
The primary treatment for choroid plexus papilloma is surgical removal. Because these tumors are usually well-defined and benign, complete surgical resection is often curative. Removing the tumor not only stops the overproduction of CSF but also restores normal fluid circulation.
In cases where hydrocephalus persists even after surgery, additional procedures such as the placement of a ventriculoperitoneal (VP) shunt or an endoscopic third ventriculostomy (ETV) may be needed to regulate fluid drainage.
Radiation therapy or chemotherapy is rarely required, since most CPPs do not behave aggressively. However, if the tumor cannot be completely removed or shows unusual features, doctors may recommend additional therapies.
Prognosis
The outlook for patients with choroid plexus papilloma is generally excellent. When the tumor is completely removed, recurrence is very uncommon, and long-term survival rates are high. The main risks are related to the complications of hydrocephalus or the surgery itself. With early diagnosis and proper treatment, many patients recover fully and go on to live normal, healthy lives.
Choroid plexus papilloma is rare, but it highlights how even benign brain tumors can cause serious symptoms because of their impact on fluid circulation in the brain. Awareness of early signs—such as persistent headaches, unexplained vomiting, or changes in head size in infants—can lead to prompt medical evaluation. With modern imaging and surgical techniques, treatment outcomes are overwhelmingly positive, offering reassurance to patients and families facing this diagnosis.
