Choroid plexus carcinoma: state of the field and emerging directions

Choroid plexus carcinoma (CPC) is a class of rare malignant life-threatening pediatric brain tumors, with a median survival of ~2.5-3 years [1, 2]. Recent advances in human genomics, cellular studies, and animal models have improved our understanding of the pathophysiology and molecular characteristics of CPC. However, because this cancer is rare, it remains understudied. This limited research has led to a limited understanding of the tumor's heterogeneity and molecular pathogenesis, resulting in fewer treatment options. Surgical resection remains the most significant contributor to patient survival and is often combined with chemotherapy, but tumors can still recur and metastasize. The post-surgical 5-year survival rate for CPC is only 60-65% and the adjuvant anti-mitotic radiation or chemotherapy included in standard treatment results in lifelong side effects for these children. Here we conduct a systematic review of existing literature and analyze the current body of research on CPC with special focus on preclinical models to highlight progress and barriers while emphasizing important open questions for improving patient treatment and longevity outcomes. This analysis underscores the importance of scholarly discussion and further exploration into the carcinogenesis of CPC.