Chordoma is a rare slow-growing malignant neoplasm thought to arise from cellular remnants of the notochord. The evidence for this is the location of the tumors (along the neuraxis), the similar immunohistochemical staining patterns, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the notochord regresses during fetal life.

Chordomas can arise from bone in the skull base and anywhere along the spine. The two most common locations are cranially at the clivus and in the sacrum at the bottom of the spine.

In the United States, the annual incidence of chordoma is approximately 1 in one million (300 new patients each year).

There are three histological variants of chordoma: classical, chondroid and dedifferentiated. The histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, sometimes described as physaliferous (having bubbles or vacuoles). Chondroid chordomas histologically show features of both chordoma and chondrosarcoma.

Prognosis and treatment

In one study, the 10-year tumor free survival rate for sacral chordoma was 46%. Chondroid chordomas appear to have a more indolent clinical course.

In most cases, aggressive surgical resection followed by radiation therapy offers the best chance of long-term control. Chordomas are relatively radioresistant, requiring high doses of radiation to be controlled. The proximity of chordomas to vital neurological structures such as the brain stem and nerves limits the dose of radiation that can safely be delivered. Therefore, highly focused radiation such as proton therapy and carbon ion therapy are more effective than conventional x-ray radiation.

There are no drugs currently approved to treat chordoma, however a clinical trial conducted in Italy using the PDGFR inhibitor Imatinib demonstrated a modest response in some chordoma patients. The same group in Italy found that the combination of imatinib and sirolimus caused a response in several patients whose tumors progressed on imatinib alone.

Notable cases

Former NFL player, Craig Heyward, was treated for a chordoma in 1995. While initially thought to be successfuly removed, the tumor returned in 2005, and caused Heyward's death in May 2006.

Josh Sommer, a student researcher at Duke University, created the Chordoma Foundation with his mother, Simone Sommer, after his diagnosis. The Chordoma Foundation has hosted two International Chordoma Research Workshops which together assembled over 100 leading scientists and physicians, many of whom were new to the field of chordoma research. The Foundation has also collected and distributed chordoma cell lines for research into the cancer's molecular cause and possible treatments.

Pro skateboarder Ray Underhill, a member of the Powell-Peralta Bones Brigade, battled chordoma for two years before succumbing to his disease in August 2008.


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