A Model for the Rarest Cancers: Choroid Plexus Carcinoma and the Li-Fraumeni Inheritable Cancer Syndrome.

In the landscape of childhood cancer, there are diseases so rare that even many physicians will never encounter a single case. Yet within these rare diagnoses lie some of the deepest biological insights and some of the most urgent clinical challenges. Choroid plexus carcinoma, often abbreviated as CPC, is one such disease. It is a malignant brain tumor that arises predominantly in very young children, most often under the age of four. Though rare, it is biologically revealing, clinically formidable, and, in recent years, the focus of a determined effort to change its outcome.