Chordoma

Specialty Area: Spine Center

Chordomas, unique malignant tumors which arise from notochordal remnants of the developing spine, can occur anywhere within the spine. Although they can appear very bland on pathology reports giving the false impression that they are benign, chordomas are actually locally aggressive tumors. They tend to be inactive for a period of time, but become quite aggressive and grow to enormous size. This growth causes localized problems and, as the disease progresses, can metastasize to other parts of the body. This can ultimately result in uncontrolled tumor and death if not treated aggressively at first presentation.

The Spine Center at Johns Hopkins has vast experience with sacral chordomas, which has allowed us to develop a comprehensive philosophy for their treatment. Because these tumors metastasize, the first operative intervention provides the best chance for cure and control of the tumor. In addition, surgical en-bloc resection provides overall increased survival and tumor control. Because chordomas are fairly rare, success is achieved best through a team approach to treatment by a highly experienced group of physicians. Treatment involves preoperative planning, surgical resection, and postoperative therapy.

Preoperative Planning
Preoperative magnetic resonance imaging (MRI) and computed tomography (CT) scans are essential to creating an individualized treatment plan. Because surgical treatment of chordomas can be aggressive and radical, it is very important that a definitive diagnosis be made prior to surgery. A physician will first screen for any cancer that has spread to other parts of the body. If the tumor hasn’t spread, they will use a needle to obtain a sample for biopsy in order to confirm the diagnosis. Because any seeding of the needle tract can cause the tumor to spread along the tract and lead to treatment failure, the team must be prepared to remove the chordoma the same day if the pathologist confirms the diagnosis.

Surgical Resection
Following the Johns Hopkins history of deep collaboration between specialties, the Spine Center takes a team approach to surgical resection. Depending on the specific needs of the patient, the team can involve neurosurgery, surgical oncology, Orthopedic Oncology, urology, vascular surgery, plastic surgery and anesthesiology.

Post Operative Care
Intensive post operative care is imperative for the success of the surgery. Patients treated for chordoma will receive care in the neurosurgical intensive care unit (NICU), physical therapy, occupational therapy, and physical medicine and rehabilitation. Radiation therapy may also be necessary. As these tumors have unique biology, currently the best mode of radiation therapy involves the use of proton beam irradiation.

Because of the high likelihood of re-growth, consistent follow up is required throughout a patient’s life. During the first year after surgery, an MRI is required every three months. In the years that follow, the time between imaging is gradually increased.