Pediatric Choroid Plexus Carcinoma: Current Management and Future Directions

Choroid plexus (CP) carcinoma is a rare intraventricular brain tumor derived from the CP epithelium and occurs mainly in young children. CP carcinoma is a World Health Organization (WHO) grade III neoplasm. Despite multidisciplinary treatment approaches, including surgical resection, radiotherapy, and adjuvant chemotherapy, the outcome remains guarded and many of the survivors exhibit significant neurologic sequelae of both the tumor and its treatment.TP53 mutations are common in CP carcinomas and have been associated with poorer outcome. Recent studies have highlighted the potential of genome-wide methylation profiling, gene expression profiling, and p53 mutation status to provide additional layers of information to improve risk stratification in CP tumors. In this chapter, we discuss the current management of CP carcinoma and future therapeutic potentials in the era of new molecular discoveries in this rare tumor. Further studies are needed to standardize the treatment paradigm for this neoplasm.