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Chordoma

Abstract

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NORD is very grateful to Justin M. M. Cates, MD, PhD, Associate Professor of Pathology, Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, for assistance in the preparation of this report.

Synonyms of Chordoma

  • Clival Chordoma
  • Familial Chordoma
  • Intracranial Chordoma
  • Sacrococcygeal Chordoma
  • Skull Base Chordoma
  • Spinal Chordoma

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
A chordoma is a rare tumor that develops in the bones of the skull and the spine. These tumors arise from the remnants of the notochord, a flexible, rod-like structure that provides support to the developing embryo. During fetal development, the notochord is replaced by the bones of the spine. Notochordal cells that persist within the spinal column may give rise to chordoma. Chordomas are slow growing tumors that cause destruction of the surrounding bone and eventually extend into the surrounding soft tissue. Sometimes chordoma spreads through the bloodstream (metastasizes) to other organs, such as the lungs, lymph nodes, liver or other bones. Although chordoma can develop at any point during life, it most commonly occurs in older adults. The symptoms associated with a chordoma depend upon the size and location of the tumor. Almost all cases of chordoma occur randomly for no apparent reason. In extremely rare cases, chordomas may develop in multiple members of the same family as a result of certain genetic risk factors (familial chordoma).

Introduction
Chordomas may be classified as a type of sarcoma. Sarcoma is a general term for a group of cancers that can affect bone or connective tissue - the tissues that connect, support and surround various structures and organs in the body. Although chordomas are primary bone tumors, they are sometimes classified as central nervous system tumors when they occur near the skull base.

Organizations related to Chordoma

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