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 neurosurgeon accesses the tumor during surgery, which is also the first step for treatment.
Chordoma treatment begins with surgery. When the entire tumor is successfully removed with surgery, patients statistically live longer and the tumor is less likely to recur, or grow back.
The type of surgery and next treatment steps depend on the location of the tumor. If your chordoma recurs, your doctor may suggest trying a chemotherapy that has worked for other types of cancer or that is part of a clinical trial. Additional surgery and radiation therapy may also be used to treat a chordoma recurrence.
After treatment, we follow patients closely with regular MRI and CT scans to catch any recurrence as soon as possible. When a chordoma grows back, it usually does so near the location of the original tumor, within five to seven years of the initial diagnosis.
In some cases, the cancer will spread to other parts of the body. Our goal is to find out if there are molecular clues to how the tumor will behave. Know that you have reason to be hopeful. This is a new and exciting time for chordoma research, and the future is bright.
Any chordoma patient, friend or family member is welcome to attend to learn about the latest in disease management and research in progress, network with others affected by chordoma and ask the physicians questions outside of the clinic. Spine Phila Pa ; 21 : - Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer ; 71 : - Current therapeutic options and novel molecular markers in skull base chordomas. Neurosurg Rev ; 35 : 1 - 13 ; discussion Current management of sacral chordoma.
Neurosurg Focus ; 15 : E9. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer ; 88 : - Chordoma: current concepts, management, and future directions. Lancet Oncol ; 13 : e69 - Chordoma: radiological-pathological correlation. Australas Radiol ; 49 : - Scintigraphic features of primary sacral tumors. J Nucl Med ; 27 : - Clin Nucl Med ; 33 : - Sacrococcygeal chordoma: report of a case and review of the literature. Surgery ; 78 : - Surgical seeding of chordomas.
J Neurosurg ; 95 : - Chordoma: the nonsarcoma primary bone tumor. Oncologist ; 12 : - Chordoma: a cytologic study with histologic and radiologic correlation. Cancer ; 93 : 40 - Skull base chordomas: correlation of tumour doubling time with age, mitosis and Ki67 proliferation index.
Neuropathol Appl Neurobiol ; 26 : - Stener B , Gunterberg B. High amputation of the sacrum for extirpation of tumors. Principles and technique. Spine Phila Pa ; 3 : - Clinicopathologic study of sacrococcygeal chordoma. Cancer ; 53 : - Operative treatment of sacrococcygeal chordoma. A review of twenty-one cases. J Bone Joint Surg Am ; 75 : - Skull base chordomas: management and results.
Neurol Med Chir Tokyo ; 46 : - ; discussion Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol ; 41 : 67 - Chordomas of the base of the skull and upper cervical spine.
One hundred patients irradiated by a 3D conformal technique combining photon and proton beams. Acta Oncol ; 44 : - Neurosurgery ; 68 : - Phase II study of 9-nitrocamptothecin in patients with advanced chordoma or soft tissue sarcoma. J Clin Oncol ; 23 : - Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer ; 72 : - Phase II study of imatinib in advanced chordoma.
J Clin Oncol ; 30 : - Br J Cancer ; 96 : - A person suspected of having a chordoma will have an initial examination that includes a detailed medical history, blood and urine tests, and a basic neurological examination to check for alertness, coordination, eye movement, muscle strength, and reflexes. Other tests, such as an ophthalmological exam, may be performed depending on symptoms. It is vital that an individual with a chordoma seek care from a highly trained, experienced multidisciplinary team, including neurosurgeons and neuro-oncologists at a major medical center where the physicians have proven skills in diagnosis and treatment.
Since symptoms develop gradually and chordomas are generally slow-growing, by the time an individual goes to a doctor with symptoms, the chordoma may be quite large. In fact, an individual with a very large chordoma may even be able to feel a lump along the spine. Also, since symptoms of a chordoma are common to other conditions, an accurate diagnosis will include extensive testing and expertise.
One of the tools used to diagnose a chordoma is an MRI scan, which uses magnetic fields and radio-frequency waves to create an image that can show the presence of a tumor and some of its characteristics, such as its size, precise location, and whether it has spread.
If the tumor has not spread, and depending on other factors such as location, a needle biopsy may be performed to collect a small sample to be examined under a microscope. If a surgical team decides to perform a needle biopsy of the tumor, they may be prepared to also immediately remove the chordoma once the diagnosis is confirmed by a pathologist.
This is because the procedure itself may cause cells to spread along the path of the needle. For this reason, clival chordomas are rarely biopsied. Reaching an accurate diagnosis is critical to ensure optimal outcome and require unique, specific tests.
Tests we offer include:. Treatment of a chordoma depends on its size and location, as well as the health of an individual. Successful treatment demands a medical team with extensive experience since removal and follow-up management of a chordoma is a delicate, complicated matter, and requires special attention to preserve neurological function.
The usual treatment options are surgery , stereotactic radiotherapy and sometimes chemotherapy. The most effective treatment for chordoma is surgery and the goal is to remove most or all of the tumor. Advanced surgical methods allow neurosurgeons to approach clival tumors through the nostrils, which requires no incision and leaves no facial scarring.
See Surgery for a Chordoma. Weill Cornell has more experience removing chordomas through the nostrils than most other centers in the world, having developed the technique here almost 20 years ago. For chordomas of the spine, neurosurgeons may use advanced minimally invasive techniques to approach and resect remove the tumor. Stereotactic radiosurgery SRS may give more rapid results than traditional radiation therapy. Stereotactic radiosurgery, which may be performed using a proton beam device or linear accelerator LINAC or with machines known as CyberKnife and Gamma Knife, is not traditional surgery.
Instead, this procedure uses a highly focused beam of radiation to target tumor cells while avoiding healthy tissue. See more about our Stereotactic Radiosurgery Program. Chemotherapy is the use of anti-cancer cytotoxic drugs to destroy cancer cells. Chemotherapy has not been shown to be very effective in treating chordomas. However, it may sometimes be used to try to control a chordoma that has recurred or spread elsewhere in the body. Rare Endocrine Tumors. Adrenocortical Carcinoma ACC.
Carcinoid Tumor. Rare Kidney Tumors. Clear Cell Renal Cell Carcinoma. Papillary Renal Cell Carcinoma. Translocation Renal Cell Carcinoma. Rare Soft Tissue Tumors. Clear Cell Sarcoma.
Desmoid Tumor. Extra-Cranial Rhabdoid Tumor. Infantile Myofibromatosis. Malignant Peripheral Nerve Sheath Tumor. NUT Carcinoma. Sclerosing Epithelioid Fibrosarcoma. Synovial Sarcoma. Rare Vascular Tumors.
Epithelioid Hemangioendothelioma EHE. Kaposiform Hemangioendothelioma. Other Rare Tumors.
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