Choroid plexus carcinoma

Choroid plexus carcinoma (CPC) is a rare, aggressive malignant brain tumor that arises from the choroid plexus, the tissue within the ventricles of the brain responsible for producing cerebrospinal fluid (CSF). It is classified as a WHO grade III tumor and represents the most malignant form of choroid plexus tumors. CPC predominantly affects young children, with the majority of cases diagnosed before the age of 5 years, and it accounts for a very small percentage of all primary brain tumors. The tumor typically develops within the lateral ventricles in children and the fourth ventricle in adults. Because the choroid plexus produces CSF, these tumors frequently cause overproduction of cerebrospinal fluid or obstruction of its normal flow, leading to hydrocephalus (abnormal accumulation of fluid in the brain). Key symptoms include signs of increased intracranial pressure such as headaches, nausea, vomiting, irritability, lethargy, and in infants, rapid head enlargement with a bulging fontanelle. Seizures, developmental regression, and focal neurological deficits may also occur. CPC has a tendency to invade surrounding brain tissue and can disseminate through the CSF to other parts of the central nervous system. Treatment typically involves a combination of surgical resection, chemotherapy, and in some cases radiation therapy. Maximal safe surgical removal of the tumor is the primary goal and is a critical determinant of outcome. Adjuvant chemotherapy regimens often include agents such as cyclophosphamide, etoposide, vincristine, and platinum-based drugs. Radiation therapy may be considered, particularly in older children and adults, though its use in very young children is limited due to neurotoxic effects on the developing brain. A notable association exists between CPC and germline TP53 mutations (Li-Fraumeni syndrome), and genetic testing for TP53 is recommended for affected patients. Despite multimodal treatment, the prognosis for choroid plexus carcinoma remains guarded, with 5-year survival rates significantly lower than those for the benign choroid plexus papilloma.

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