Choroid plexus carcinoma (CPC) is a challenging form of brain cancer. The tumor is most common in infants under the age of one (too young for radiation therapy). Only 40% of the infants stay alive five years following the diagnosis, and those who live through it, many a time, undergo long-term suffering from the therapy.
Scientists at Sanford Burnham Prebys have come up with a new mouse model of CPC to drug complexes with biological activity that may be useful.
“CPC is an incredibly aggressive cancer that does not respond well to treatment, but an inability to study the tumor in a controlled laboratory environment has stalled efforts to find effective drugs,” stated Robert Wechsler-Reya, Ph.D., the senior author of the paper, professor and director of the Tumor Initiation and Maintenance Program at Sanford Burnham Prebys. “This model is a valuable tool that will increase our understanding of the biology of the cancer and allow us to identify and test novel approaches to therapy. This advance brings us one step closer to a future where every child survives—and thrives—after diagnosis with CPC.”
CP tumors originate from a brain structure that produces cerebrospinal fluid, known as the choroid plexus. Cancer occurs in 3 forms. Two of them are usually resolved by tumor removal. The third, CPC, needs radiation and chemotherapy, provided the child is aged enough. In spite of these therapies, the tumor frequently returns and surviving patients go through lasting impacts from the therapies.
The mouse model was made by triggering an oncogene called Myc(able to cause cancer) and removing the p53 tumor suppressor gene in brainstem cells. These tumors imitated human cancer. Both the cancers had different expression of genes taking part in the cell cycle, reaction to DNA damage and cilium function.
“We actually set out to develop a mouse model of a different (but deadly) pediatric brain cancer called medulloblastoma. Further study revealed that the mice actually developed CPC, not medulloblastoma,” Wechsler-Reya stated. “This unexpected discovery provides many new insights into the biology of the cancer…”
The model allowed the scientists to discover the existence of 3 compounds that decreased the production of the cancer cells, without damaging brain cells(healthy), was discovered: dinaciclib, flavopiridol, and triptolide (natural).
“Our laboratory plans to evaluate these and additional compounds that can effectively treat this cancer,” told Wechsler-Reya.