Surgery for chordoma

Surgery is typically the main treatment for newly diagnosed chordoma and can be one option for recurrent, advanced, and metastaticWhen tumors have spread to other parts of the body from the original tumor site. chordoma as well. No matter what stage of the disease you are facing or where your tumor is located, the way surgery is performed is very important.^{1 2} Here are three things to know before having surgery:

Any tumor cells left behind after surgery can re-grow. Because of this, the entire tumor should be removed whenever possible, ideally with wide margins of healthy tissue surrounding the tumor. Completely removing the tumor lowers the risk of the tumor coming back after treatment.
For most patients, radiation therapy is recommended after surgery and can sometimes be recommended both before and after surgery. The outcomeThe health condition of a person after being diagnosed and treated for a particular disease. Outcome is usually measured at different time points. For example: one-, five-, or ten-year outcome. of your surgery can affect how well the radiation can be delivered, so plans for surgery should be made with the input of a radiation oncologist.
Because chordomas tend to be located near important structures, surgery can cause serious side effects. Additionally, multiple surgeries over time increases the risk of serious side effects. Before surgery, you should ask your surgeon about the risks of surgery and what to expect afterwards. If you are not comfortable with the likely side effects of surgery, ask your doctors what other options you have. You should be comfortable with the treatment plan agreed on by you and your medical team.

Margins for chordoma surgery

You will probably hear the term margins or surgical marginsThe healthy tissue surrounding the tumor that is taken out along with the tumor to make sure that no cancer cells are left behind. Negative or wide margins mean no tumor cells can be detected in the healthy tissue, which lowers the chance of recurrence. when your surgeon plans your treatment. Margins refer to the healthy tissue surrounding the tumor that is removed along with the tumor. Surgeons take out this healthy tissue to help make sure microscopic cancer cells are not left behind. The size of the margin can determine how likely the tumor is to come back after surgery. Levels of surgical margins described by the Chordoma Global Consensus GroupA multidisciplinary, international group of over 60 doctors who have extensive experience caring for chordoma patients. The group is responsible for developing and publishing consensus guidelines, based on all available medical and scientific evidence, for the treatment of primary and recurrent chordoma.^{1 2} are:

Wide resectionRemoval of the entire tumor with at least 1 millimeter of healthy tissue surrounding the tumor. – The entire tumor is removed along with at least 1 millimeter of healthy tissue around the tumor.
Marginal resectionLess than 1 millimeter of healthy tissue removed along with the tumor but no visible tumor tissue left behind. – Less than 1 millimeter of healthy tissue around the tumor is removed, but no visible tumor tissue left behind.
Intralesional resectionVisible tumor tissue has been left behind or tumor cells have spilled into the surrounding area because the tumor was cut during surgery. – Visible tumor tissue is left behind, or tumor cells have spilled into the surrounding area because the tumor was cut during surgery.

A wide resection is the best outcome, whenever possible. However, the location of chordomas near important structures sometimes makes wide resection difficult or impossible.^{1 2}

Questions to ask about treatment

To help you get the best care possible, we developed lists of questions with the input of chordoma patients and caregivers that can help you think about what you want to ask your doctors. View question lists »

Skull base tumors

Surgery should be performed in a medical center with substantial experience in skull baseThe bones at the bottom part of the skull that separate the brain from other structures. The clivus is one of the bones of the skull base. surgery. Skull base operations are usually done by a team that includes a neurosurgeon and an ear, nose, and throat (ENT) surgeon. ENT surgeons are also called otolaryngologists.^{1 2} Surgery to remove skull base chordomas can either be done endoscopicallyUse of an instrument to look inside the body. Most skull base chordoma surgeries are done endoscopically through the nose., using a small scope through the nose or mouth, or through the side of the head. Depending on the size and location of your tumor, it may be necessary to do surgery from multiple directions to safely remove different parts of the tumor. Ask your surgeon about different surgical approaches that are available to you, and discuss the risks and benefits of each. For suspected cases of skull base chordoma, biopsies are often performed at the time of surgery to help reduce the risks involved. In some cases, a pathologist will examine a sample of tumor tissue at the beginning of surgery so that the surgeons can proceed with surgery knowing what type of tumor they are removing.

The goal of surgery for skull base tumors is complete resectionAll visible tumor has been removed, but not necessarily in one piece., or complete removal of all visible tumor tissue, whenever possible. Published medical studies have shown that complete resection of skull base chordomas is associated with lower rates of recurrenceTumor that has grown back after initial treatment. Recurrences can be isolated or multifocal, local or regional. and higher rates of survival.^{3 4 5} Skull base chordomas often touch important nerves and blood vessels, so it is not always possible to remove the entire tumor. Even in cases where all visible tumor can be removed, it is still likely that microscopic chordoma cells will be left behind after surgery. Therefore, radiation therapy is generally recommended after surgery to prevent any remaining cells from re-growing or spreading to other parts of the body.^{1 2} If the entire tumor cannot be removed, your surgeon should remove as much of the tumor as possible, especially around the brainstemThe brainstem is the lower part of the brain connected to the spinal cord. The brainstem relays all signals to and from the brain and the body and is responsible for maintaining consciousness, breathing, and heartbeat. and optic nerveThe nerve responsible for eyesight by sending signals from the eye to the brain., so that radiation therapy can be most effective. Read more on the Radiation for chordoma page of our website.

Learn about the Chordoma Global Consensus Group guidelines for newly diagnosed skull base chordoma »

Learn about the Chordoma Global Consensus Group guidelines for recurrent skull base chordoma »

Mobile spine and sacral tumors

Surgery should be performed in a medical center with substantial experience removing tumors of the spine and sacrumThe five vertebrae at the base of the spine near the pelvis, and between the two hip bones. These bones are commonly referred to as S1-S5.. These centers have teams of surgeons that can include specialties such as neurosurgery, orthopedic surgery, plastic and reconstructive surgery, and colorectal surgery.^{1 2} The goal of surgery for mobile spineThe parts of the spine not including the sacrum. The cervical spine (neck), thoracic spine (upper back), and lumbar spine (lower back) are the parts of the mobile spine. and sacral chordomas is to completely remove the tumor in one piece with wide margins of normal tissue surrounding it.^{1 2} This is called en-bloc resectionDuring surgery, removal of the entire tumor in one piece without cutting it into smaller pieces.. Removing the tumor in more than one piece or rupturing the tumor during surgery can spill tumor cells into the area which can cause the tumor to re-grow or spread.^{6 7 8} Medical studies of patients who had surgery for a primary (newly diagnosed) chordoma have shown that complete resection with wide, tumor-free margins is associated with lower rates of recurrence and higher rates of survival.^{6 7 8} If you had a biopsyA procedure that uses a needle to remove a small tissue sample from the tumor to be tested in order to make a diagnosis., tissue that was touched by the biopsy needle should be removed so that any tumor cells that may have been left behind do not grow. Once the tumor is removed, reconstructive surgery may be needed to repair or replace tissue lost during surgery. Metal hardware or bone grafts are often used to replace bones that were destroyed by or had to be removed with the tumor. In some cases, surgery can cause serious side effects. Sacral patients can experience loss of bowel and bladder control, sexual function impairment, neuropathyChronic pain caused by nerve damage., and movement problems. Mobile spine patients can experience swallowing issues (cervical spineThe seven vertebrae that make up the neck. These vertebrae are commonly referred to as C1-C7.), mobility issues, and neuropathy. Surgeons can usually predict how severe these side effects will be depending on the location of the tumor and which nerves are involved. Radiation is generally recommended along with surgery for mobile spine and sacral tumors, either after surgery or both before and after surgery.^{1 2} Read more on the Radiation for Chordoma page of our website. Talk with your doctors about your options to decide what treatment plan is best for you.

Learn about the Chordoma Global Consensus Group guidelines for newly diagnosed mobile spine and sacral chordoma »

Learn about the Chordoma Global Consensus Group guidelines for recurrent mobile spine and sacral chordoma »

What to expect after surgery

Each patient’s recovery will differ depending on the size and location of the tumor, extent and difficulty of the surgery, and what nerves have been affected. Skull base patients may need rehabilitation services such as physical therapy for speech, vision, or swallowing issues. Patients with mobile spine or sacral tumors will very likely need rehabilitation therapy following surgery to help with any functional loss or mobility issues caused by the tumor or the surgery. Pain is common for most patients and can be quite severe. Specialties to help with these issues include:

Physiatry
Physical therapy
Occupational therapy
Pelvic floor therapy
Ostomy management
Pain management

If radiation treatments are planned after surgery, these will begin once you have recovered from surgery. This could be anywhere from a few weeks to a few months, but unless there are unusual complications radiation treatments will typically begin within six months.

Learn more about radiation for chordoma »

Can palliative care help?

Palliative careCare given to improve the quality of life of patients who have a serious or life-threatening disease. is very specialized care meant to help patients have the best quality of life possible while living and dealing with a serious illness or disease. A palliative care specialist can help patients and caregivers learn about, engage, and coordinate the services of different specialties – like those listed above – that may be needed to manage pain and any other side effects. It is recommended that all chordoma patients seek the services of a palliative care team from the time of diagnosis through all stages of treatment and recovery.

Follow-up after treatment

Following any treatment for chordoma, it is very important to maintain a regular schedule of MRIs and checkups with your doctor. These follow-ups will monitor your recovery, assess ongoing quality of life needs, as well as check for new tumor growth.

Learn more about the recommended follow-up schedule for chordoma »

References and further information

Stacchiotti S, Sommer J, Chordoma Global Consensus Group. Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncology. 2015 Feb;16(2):e71-83. doi: 10.1016/S1470-2045(14)71190-8.
Stacchiotti S, Gronchi A, Fossati P, et al. Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 2017 Jun 1;28(6):1230-1242. doi: 10.1093/annonc/mdx054.
Koutourousiou M, Snyderman CH, Fernandez-Miranda J, Gardner PA. Skull base chordomas. Otolaryngol Clin North Am. 2011 Oct;44(5):1155-71. doi: 10.1016/j.otc.2011.06.002. Epub 2011 Jul 21.
Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncology. 2012 Feb;13(2):e69-76. doi: 10.1016/S1470-2045(11)70337-0.
Di M, Temkin N, Ramanathan D, Sekhar L. Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies. J Neurosurg. 2011 Dec;115(6):1094-105. doi: 10.3171/2011.7.JNS11355. Epub 2011 Aug 5.
Sciubba DM, Cheng JJ, Petteys RJ, Weber KL, Frassica DA, Gokaslan ZL. Chordoma of the sacrum and vertebral bodies. J Am Acad Orthop Surg. 2009 Nov;17(11):708-17.
Bergh P, Kindblom L, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom J. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000;88(9):2122-2134. [PubMed]
Boriani S, Bandiera S, Biagini R, et al. Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976). 2006;31(4):493-503. DOI: 10.1097/01.brs.0000200038.30869.27.

The information provided herein is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your or your loved one’s physician about any questions you have regarding your or your loved one’s medical care. Never disregard professional medical advice or delay in seeking it because of something you have read on this Website.

Chordoma Foundation