ABSTRACT
Choroid plexus tumors are rare intraventricular neoplasms that encompass three histological subgroups: choroid plexus papilloma (CPP), atypical choroid plexus papilloma (ACPP), and choroid plexus carcinoma (CPC). These are primary brain tumors originating from the epithelial cells of the choroid plexus. Choroid plexus tumors are rare intraventricular papillary neoplasms, accounting for approximately 2"–4" of intracranial tumors in children and 0.5" in adults. Although a majority of choroid plexus tumors arise sporadically, some are linked to hereditary factors. Differential diagnosis of CPP includes both normal choroid plexus and villous hypertrophy of the choroid plexus, papillary ependymoma, and astroblastoma. Gross total resection is usually curative for CPP and ACPP; adjuvant radiotherapy and/or chemotherapy may be required for CPC and incompletely resected ACPP. CPC carries an extremely poor prognosis, with a 5-year overall survival rate of 36" after surgery and long-term cognitive and developmental deficits in surviving patients.
