Understanding the Pathophysiology of Chordomas

Sagar R. Shah, M.S.

PhD Candidate, Dept. of Biomedical Engineering
National Science Foundation Graduate Research Fellow

Introduction

Chordomas are tumors that arise from the osseous spine and skull base and comprise 2-4% of all bone cancers. They are often refractory to treatment with a median survival of approximately 6 years. Recent reports on the incidence and survival patterns of chordoma patients underscore the dismal prognosis of this disease with long-term survival rates at 5, 10 and 20 years precipitously dropping to 67%, 40% and 13%, respectively. Like gliomas and other aggressive malignancies of the neuroaxis, the invasive nature of this cancer often involves critical neurovascular structures of the spine and skull base making complete surgical resection impossible in 50% of the cases of sacral chordomas and even more difficult in the mobile spine and skull base.

Aims/Goals

Our long-term goal is to understand the biology of chordomas and the molecular aberrations these rare tumors harbor.
Current Progress: Recently, we reported the generation and characterization of a primary chordoma cell line. Currently, we are investigating the role of certain genes that have been previously been implicated in the pathogenesis of these tumors.

Clinical Implications

Understanding the molecular landscape of these tumors will enable us to design appropriate and targeted therapeutic agents to target this malignancy. In addition, much of the knowledge gained from these studies will have clinically-relevant implications in other tumors of the central nervous system such as gliomas.