Choroid plexus tumor

Choroid plexus tumors (CPTs) are rare neoplasms arising from the choroid plexus epithelium, the tissue responsible for producing cerebrospinal fluid (CSF) within the ventricles of the brain. These tumors account for approximately 0.3–0.6% of all intracranial tumors and 2–4% of brain tumors in children. They are classified into three subtypes based on the World Health Organization (WHO) grading system: choroid plexus papilloma (WHO grade I, benign), atypical choroid plexus papilloma (WHO grade II, intermediate), and choroid plexus carcinoma (WHO grade III, malignant). The tumors predominantly occur in the lateral ventricles in children and in the fourth ventricle in adults. The primary clinical manifestation of choroid plexus tumors is hydrocephalus, caused by overproduction of CSF, obstruction of CSF flow, or impaired CSF absorption. This leads to symptoms of increased intracranial pressure, including headache, nausea, vomiting, irritability, lethargy, and in infants, rapid head enlargement (macrocephaly) and bulging fontanelles. Seizures, visual disturbances, and developmental delays may also occur. Choroid plexus carcinomas may invade surrounding brain tissue and, rarely, metastasize through the CSF pathways. Treatment typically involves surgical resection, which is the primary therapeutic approach and can be curative for choroid plexus papillomas when complete removal is achieved. For atypical papillomas and carcinomas, adjuvant chemotherapy and/or radiation therapy may be necessary, particularly when complete surgical excision is not possible or when there is evidence of dissemination. A subset of choroid plexus carcinomas has been associated with Li-Fraumeni syndrome caused by germline mutations in the TP53 gene, and genetic testing is recommended, especially in young children with choroid plexus carcinoma. Prognosis varies significantly by tumor grade, with choroid plexus papillomas having an excellent prognosis after complete resection, while choroid plexus carcinomas carry a more guarded outcome.

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