Primary Choroid Plexus Carcinoma of the Fourth Ventricle in an Adult: A Case Report and Literature Review

Abstract According to the latest WHO classification (5th edition), choroid plexus carcinoma (CPC) is a rare, grade 3 malignancy originating from the choroid plexus, predominantly affecting pediatric patients. In adults, CPC is uncommon, and the limited data available have hindered the development of standardized treatment protocols. We reviewed the literature to evaluate the clinical behavior and treatment outcomes of CPC in adults. Additionally, we performed a statistical analysis of the available data and we present a case of an adult female patient treated at our institution. From the literature, we identified 36 adult patients aged 18 to 73 years. CPC was primarily located intraventricularly and supratentorially, though other locations were noted. Gross total resection (GTR) was achieved in 21 patients, while 15 underwent partial resection (PR). Adjuvant radiotherapy (RT) was administered in 16 cases, chemotherapy (CTx) in 1, a combination of RT/CTx in 10, and stem cell transplantation in 1. A significant correlation was observed between resection type and survival, though no significant associations were found with gender, adjuvant therapy, age, or tumor location. CPC in adults carries a poor prognosis, and definitive diagnosis relies on histopathology. Treatment typically involves surgical resection, while adjuvant therapies offer limited benefit to overall survival (OS). There is currently no consensus on a standard treatment approach, and outcomes remain unsatisfactory. Ongoing research is crucial for improving the understanding and management of this entity.