Papilloma of choroid plexus

Papilloma of the choroid plexus (also known as choroid plexus papilloma, or CPP) is a rare, typically benign brain tumor (WHO grade I) that arises from the epithelial cells of the choroid plexus, the tissue within the brain's ventricles responsible for producing cerebrospinal fluid (CSF). This tumor primarily affects the central nervous system and is most commonly diagnosed in young children, particularly during the first two years of life. In children, the tumor most frequently occurs in the lateral ventricles, while in adults it tends to arise in the fourth ventricle. Choroid plexus papillomas account for approximately 0.4–0.6% of all intracranial tumors and about 2–4% of brain tumors in children. The key clinical features result primarily from overproduction of CSF and obstruction of CSF flow, leading to hydrocephalus (abnormal accumulation of fluid in the brain). Patients commonly present with signs and symptoms of increased intracranial pressure, including headache, nausea, vomiting, irritability, and in infants, an enlarging head circumference (macrocephaly) and a bulging fontanelle. Visual disturbances, papilledema, gait abnormalities, and seizures may also occur depending on tumor location and size. The primary treatment for choroid plexus papilloma is surgical resection, which is often curative when complete removal is achieved. The prognosis is generally excellent, with high long-term survival rates following gross total resection. Recurrence is uncommon after complete surgical excision. In cases where hydrocephalus persists after tumor removal, CSF diversion procedures such as ventriculoperitoneal shunting may be necessary. Chemotherapy and radiation therapy are generally not required for benign choroid plexus papillomas but may be considered in the rare event of malignant transformation to choroid plexus carcinoma (WHO grade III). Regular follow-up imaging is recommended to monitor for recurrence.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Papilloma of choroid plexus