Chordoma

A chordoma is a rare, cancerous tumor that can grow anywhere along your spine, most often in the bottom set of vertebrae called the sacrum. A type of bone sarcoma, chordomas represent less than four percent of bone cancers and occur most often in people ages 40 to 70.

Chordomas appear to come from the notochord, a collection of cells that guides spine formation while a baby develops in the womb. Occasionally, pieces of the notochord stay in the body after the spinal bones fully develop. In some cases, those pieces don’t cause any problems and just need to be watched. In other cases, they turn into cancer for reasons not yet fully understood.

It’s possible to develop more than one chordoma tumor at a time in different places along the spine. These tumors are slow growing but can still damage surrounding bone and invade nearby soft tissues. They also metastasize (spread) in 30 percent of cases, usually to the lungs.

The Sarcoma Program at Penn Medicine’s Abramson Cancer Center is home to one of the nation’s most experienced chordoma care teams. Our specialists treat hundreds of people with sarcomas each year, including chordomas. We also have a spinal tumor program with a multispecialty team of experts who provide timely diagnosis and specialized treatment of spinal tumors. After treatment, you’ll receive important follow-up monitoring from your care team so that any recurrent chordomas are addressed right away, helping you manage your health over the long-term.

In most cases, doctors don’t know why cancer develops from leftover notochord. Researchers continue to investigate this question, but finding answers is challenging because chordomas are so rare.

Unlike some other bone cancers, there is no evidence that chordomas have ties to previous medical treatments. However, some children who have a rare genetic condition called tuberous sclerosis develop chordomas, along with a range of other tumors. Sometimes tuberous sclerosis is inherited from a parent. At other times, the genetic changes responsible take place in the womb.

Chordoma treatment is complex and requires an experienced team to evaluate the tumor and recommend the best course of action. At Penn Medicine, we have a history of performing research and conducting clinical trials on a wide range of sarcomas, including chordomas. This means we bring deep knowledge and experience to your care and offer the latest evidence-based treatments.