Surgery for chordoma
Surgery is typically the main treatment for newly diagnosed chordoma and can be one option for recurrent, advanced, and metastatic 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 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.
You will probably hear the term margins or surgical margins 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 Group1 2 are:
- Wide resection – The entire tumor is removed along with at least 1 millimeter of healthy tissue around the tumor.
- Marginal resection – Less than 1 millimeter of healthy tissue around the tumor is removed, but no visible tumor tissue left behind.
- Intralesional resection – 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.
Skull base tumors
Surgery should be performed in a medical center with substantial experience in 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 endoscopically, 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 resection, 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 recurrence 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 brainstem and optic nerve, so that radiation therapy can be most effective. Read more on the Radiation for chordoma page of our website.
Mobile spine and sacral tumors
Surgery should be performed in a medical center with substantial experience removing tumors of the spine and sacrum. 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 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 resection. 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 biopsy, 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, neuropathy, and movement problems. Mobile spine patients can experience swallowing issues (cervical spine), 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.
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:
- 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.
Can palliative care help?
Palliative care 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.
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.