Q&A: Chordoma, a one in a million bone cancer

When you have a rare cancer that only affects one in a million people, it can be difficult to find the answers and information you need. That’s why we sat down with spinal neurosurgeon Laurence Rhines, M.D., and skull base neurosurgeon Shaan Raza, M.D., to discuss chordoma, a rare, malignant tumor diagnosed in about 300 people each year in the United States. What is chordoma? Chordoma is a bone cancer that belongs to the sarcoma family. About 50% of chordomas occur in the sacrum, the bone at the base of the spine that forms part of the pelvis. About 30% occur in the skull base, the area behind the eyes and nose that slopes down to form the “floor” of the skull. The rest are found along the spine. What are common chordoma symptoms? The symptoms vary, depending on the tumor’s location. The spine and skull base contain important nerves. When a tumor presses on these nerves, it can cause pain or neurological issues. For sacral chordomas, neurological symptoms frequently include bowel, bladder and sexual dysfunction. For skull base chordomas, common symptoms include double vision, headaches, neck pain and trouble swallowing. How is chordoma diagnosed? An MRI scan can help rule out other tumor types, but a tissue sample is needed for a definitive chordoma diagnosis. If your tumor is in the spine, an interventional radiologist will typically perform a CT-guided core biopsy to obtain a tissue sample. Most skull base chordomas grow in a bone called the clivus. This area is difficult to access for biopsy, so a skull base chordoma diagnosis cannot be confirmed until after your...