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The Massachusetts General Hospital Cancer Center’s Center for Sarcoma and Connective Tissue Oncology provides patients with expert chordoma care. We bring a unified focus to the evaluation, treatment, and support of chordoma patients, and to research advancing understanding of the causes of and treatments for this rare cancer.
Massachusetts General Hospital Cancer Center is home to the new Stephan L. Harris Center for Chordoma Care, a comprehensive treatment and research center. In addition, the availability of proton beam therapy, as well as expertise in surgical management of spinal tumors, attract chordoma patients from around the world. Collaboration with specialists from the Center for Head and Neck Cancers, the Cranial Base Program, and the Massachusetts Eye and Ear Infirmary ensures expert, coordinated care for the widely varying manifestations of chordoma.
Our musculoskeletal pathologists specializing in cancer of the bones and soft tissues consult on chordoma cases from around the world. Every biopsy is carefully evaluated to ensure an accurate diagnosis and to exclude other tumors that can mimic chordomas.
During surgery, the pathologist works closely with the surgeon and radiation oncologist to make sure that the resection is as effective as possible. The archiving of tissue collected at the time of biopsy and resection is enabling our pathologists to study chordomas on a molecular level. Their research is aimed at discovering the pathogenesis of chordomas and uncovering tumor markers to guide future therapies.
Advanced imaging technologies help to define the size and shape of the tumor, identify important normal tissue nearby, and aid in removal of the tumor and protection of normal tissue. These include:
The Stephan L. Harris Center for Chordoma Care at Mass General provides extraordinary, compassionate care while advancing the art and science of evaluating, diagnosing, treating and supporting patients. Treatment is tailored to address the unique needs of each patient, and usually involves advanced surgical or radiation techniques, or a combination of these approaches.
Our surgeons have extensive experience treating chordoma while minimizing damage to surrounding structures. Advanced surgical techniques include:
Our radiation oncologists offer a range of therapies designed to shrink or destroy tumors while protecting normal tissue. Advanced technologies include:
Learn more about radiation therapy.
Physician-scientists in the Sarcoma Molecular Biology laboratory are pursuing research to identify the genetic abnormalities associated with chordoma. We have the largest archive in the world of chordoma tissue, which we study to uncover the mechanisms that drive chordoma and to search for a cure.
Learn more about chordoma research under way at Mass General's Harris Center.
Our team approach ensures that every patient benefits from an individualized, coordinated and compassionate treatment plan, with care provided by leading experts from many specialties. We consider patients and their family and friends involved in their care to be part of this team.
Your care team may include:
Chordoma is a rare malignant bone tumor. A group of cells known as the notochord come together during the fourth through six weeks of fetal development. The notochord serves as the base for the development of the spinal cord. As the spinal cord forms, the notochord disappears, except for parts that form part of the discs between the vertebrae. Chordomas form from parts of the notochord that don’t break down as they should. Over time these leftover cells can change and become malignant. Harris Center researchers are studying these changes to develop new therapies for chordoma. Chordoma primarily spreads by local invasion, but it does have the potential to spread to the lungs, bone and liver.
Most develop at the base of the skull, the base of the spine or tailbone, but they can occur in other areas of the spine. Ttumors at the base of the skull most commonly appear between the ages of 35-40. Those that arise from the sacrum usually appear between the ages of 50 and 70. Chordoma can occur in children but it is more common in adults. Approximately 300 new cases of chordoma are diagnosed in the US each year. Chordoma occurs more frequently in men. There are no known risk factors for chordoma.
Pain and changes in sensation or function are the most common symptoms of chordoma. Tumors located at the base of the skull may cause headache, neck pain, difficulty swallowing, or double vision. Chordoma in other areas of the spine may cause pain, numbness or weakness in the arms or legs. Changes in bowel or bladder function may occur.
The symptoms are similar to those caused by other disorders and chordoma grows very slowly, so patients may have symptoms for more than a year before seeking treatment.
Abnormalities are usually evident during a neurological examination, but imaging studies are needed. Magnetic Resonance Imaging (MRI) is the most useful test. In addition, a Computed Tomograhy (CT) study may be done to rule out spread of chordoma to other areas of the body. To make the final diagnosis, a biopsy is usually required.
Because Chordoma develops close to vital structures in the central nervous system, treatment is complex and can be difficult, but the prognosis is not always grim. In a recent study, Harris Center clinicians achieved a five-year continuous disease-free survival rate of 62.5% with primary sacral chordoma.
Click here for the PubMed summary of a recent paper by Harris Center clinicians on the efficacy of chordoma surgery performed in concert with proton-beam therapy.
The primary treatment for chordoma is the complete surgical removal of the tumor. To ensure the best outcome and prevent the spread of chordoma , the surgeon(s) must remove the chordoma and a margin of normal tissue surrounding it. The result of the first surgery is critical in determining the patient’s prognosis (outcome) It is vital that an experienced, expert team of surgeons perform the initial operative procedure
Skull based chordoma are generally removed through minimally invasive surgery by highly trained, skilled surgeons. Patients with chordoma at the base of the skull following this procedure usually require a brief hospital stay.
Chordomas that occur in the cervical, thoracic, lumbar or sacral spine have a larger area to grow requiring a more extensive operative procedure to remove them.
At the Harris Chordoma Center the surgeons perform the operative procedure in one or two stages depending on the size and location of the tumor. The surgeons are acutely aware of the risks in chordoma surgery. Every precaution is taken to avoid spread of tumor in the surgical wound. The use of preoperative radiation therapy by Harris Chordoma Center radiology oncologists helps to avoid this complication.
Sacral chordomas, those in the lower spine, may involve nerves that control the bowel and/or bladder function. Most patients are able to manage control using a bowel regime and/or intermittent catherization. A colostomy may be indicated if the size and location of the chordoma requires the sacrifice of nerves that control bowel function.
The multidisciplinary approach continues throughout the patient admission including general surgical oncologists; thoracic surgeons; ear, nose and throat specialists (ENT); and reconstructive surgeons; neurological surgeons; orthopaedic oncology surgeons; radiation oncologists; orthopaedic pathologists; medical oncologists; and clinical nurse specialists; social workers; physical therapists; and occupational therapists working as a disciplined team to achieve the best possible outcome for the individual patient
Surgical intervention for the removal and treatment of cervical, thoracic, lumber or sacral chordoma by and large requires a hospital admission of 3-10 days and likely follow-up rehabilitation in a hospital or specialty facility.
Pre and/or postoperative radiation therapy has improved local control of chordoma. It can also slow the growth of the tumor if it has been incompletely resected or if it has recurred.
Proton beam therapy uses energy from protons of atoms to destroy cancer cells. It can be aimed at a tumor very precisely and can be concentrated on the tumor with little harm to the surrounding tissue.
For some patients for whom surgery is not a good option, high-dose radiation with a combination of x-rays and protons has been able to control tumors for over a decade and may be an important option for them.
Click here for information on proton beam therapy at Mass General Hospital
Chemotherapy has not proved effective in the past, but there are a number of clinical trials with new agents currently underway.
The Cancer Center is a site for trials of new targeted agents as well as a National Institutes of Health-sponsored study of familial chordoma. We can offer participation in this study to families in which two or more relatives have chordoma. Our goal is to search for genetic or hereditary factors that may help us to better understand the disease and suggest new therapies.
Phone: (617) 726-5130
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