Choroid Plexus Carcinoma in Adults: Literature Review and First Report of a Location into the Third Ventricle

Abstract

Choroid plexus carcinoma (CPC) is a rare intraventricular neoplasm originating from choroid plexus. CPC is the most aggressive choroid plexus tumor. Almost all the CPCs are detected in children, and the preferred location is the lateral ventricle. We reviewed the literature to evaluate the state of the art concerning the management of CPC in adults and report the first case described of the extremely rare localization into the third ventricle. A 38-year-old woman presented a medical history of Parinaud syndrome and occasional facial weakness. Brain magnetic resonance imaging revealed a mass lesion in the pineal region and posterior part of the third ventricle with obstructive hydrocephalus. She underwent subtotal resection through a supracerebellar infratentorial approach. After the histopathological diagnosis of CPC, the patient underwent a second surgery with gross total resection and adjuvant radiotherapy. CPC in adults, given its extreme rarity, does not have a standardized treatment. Gross total resection should be the first step of the treatment: however, according to the literature, gross total resection is achieved only in 40-75% of cases in CPC as opposed to 95% in choroid plexus papilloma, mainly due to the difficulty in managing a highly vascularized tumor in such a deep location. Chemotherapy has not an established role and adjuvant treatment is based on radiotherapy. In the case described hereby the gross total resection associated with surgical treatment of hydrocephalus and adjuvant radiotherapy has achieved a good clinical and radiological outcome.

Keywords: Carcinoma; Choroid plexus; Gross total resection; Papilloma; Third ventricle.