Chordomas and Surgery

Chordoma is a rare, malignant cancer often found at the base of the skull or bottom of the spine. Although surgery is the primary treatment option, the deep location of the tumor can make complete removal challenging. The goal of surgery is to remove as much of the tumor as possible while preserving normal structures nearby. In some cases, a combination of surgery and radiation therapy may be performed to help prevent recurrence or slow the growth rate.

Upon reviewing the images of your skull base chordoma with your medical team, the thought of surgery can seem scary and even impossible. Neurosurgeons, particularly those specialized in skull base techniques, are equipped with the knowledge and skills to access this challenging tumor. The surgery may be performed using one of two common approaches: endoscopic or open surgery.

An endoscope is a long, thin handheld camera that allows your surgeon to visualize small spaces from relatively far distances away. This is particularly useful when attempting to access the tumor through the nose in what is known as a transnasal transsphenoidal approach. In this operation, your surgeon will make small incisions inside of your nose, drill off the bone at the base of the skull, then remove the tumor using long surgical instruments. While performing this approach, the surgeon uses his other hand or another assistant to operate the endoscope. This surgery is minimally invasive and has a lower risk of complications than traditional open surgery. Generally, no scars are externally visible. Following surgery, you will be prescribed medications to help control any pain and be monitored for a few days at the hospital.

In some cases, the chordoma cannot be accessed through the nose because of its large size or location. That is when your neurosurgeon will suggest a traditional open surgical approach to remove the tumor. This involves a procedure called a craniotomy, where a piece of your skull bone is removed temporarily to access the brain and tumor.

The first step in this approach is to make an incision in the scalp. Next, the surgeon will remove a portion of the skull using specialized tools. Once the brain is exposed, special surgical instruments are used to gently retract the brain and find the tumor. The tumor is then removed while normal neighboring nerves, blood vessels, and brain tissues are preserved.

Oftentimes, complete removal of the tumor is impossible because of the diffuse nature of the tumor itself, and because of its proximity to critical structures. However, once a large portion of the tumor is removed, the bulk of the tumor is smaller and farther away from surrounding tissues. Radiation therapy may then be more safely administered to the tumor bed to slow growth.

Once the chordoma is removed, the surgeon will replace the skull and secure it with plates and screws. Finally, the scalp is closed with sutures or medical staples and the incision is dressed. You will then wake up from anesthesia and be moved to the recovery room for close monitoring.

If you had endoscopic surgery, you may have some pain and discomfort in the nose and throat. Limit excessive blowing of your nose, sneezing, or coughing for a few weeks after the surgery. Physical activities such as bending, lifting, or strenuous exercises should be avoided. A high-fiber diet rich in fruits and vegetables can help to prevent constipation and promote overall recovery.

If you received open chordoma surgery, you may have some pain, swelling, and numbness around the surgical site. Not to worry — this is normal. Any sutures are usually removed 7 to 10 days following the surgery.

Remember, every case is different, and the recovery process may vary from person to person. If you have any concerns or questions about your recovery, don’t hesitate to reach out to your surgeon.

One type of surgery that is often used to treat sacral chordomas is called a wide resection. It involves removing the tumor and some healthy tissue around it. This can help ensure that all the cancer cells are removed.

Another type of surgery used to treat sacral chordomas is called a sacrectomy. It involves removing a portion of the bony sacrum along with the tumor. Sacrectomy is often used when the tumor is very large or difficult to reach. In some cases, sacral reconstruction procedures may also be done.

After either surgery, you may need to undergo radiation therapy to help eliminate any remaining cancer cells.

With any surgery, there is a risk of bleeding, infection, and injury to surrounding important structures. Other possible complications of surgery for skull base chordomas include:

• Visual disturbances such as double vision or loss of vision
• Damage to the pituitary gland, which can lead to hormonal imbalances
• Recurrence of the tumor, which may require additional treatments such as radiation therapy or chemotherapy
• Leak of cerebrospinal fluid (CSF), a watery colorless fluid that surrounds the brain, from the nose or surgical site; this can cause headaches, nausea, and infection

Some potential risks of sacral chordoma surgery include:

• Urinary retention: This occurs due to damage to the nerves that control urination and may require the insertion of a catheter to drain the urine.
• Bowel movement disturbances: If the tumor has encased the nerves that control bowel movements, removing the chordoma may damage these nerves and cause difficulty with controlling bowel movements.
• Difficulty walking: Because the sacrum plays an important part in walking, surgery for sacral chordoma may result in changes in posture and your gait.
• Sexual dysfunction: As the nerves that assist with sexual function are in the sacrum, they may get injured during sacral chordoma surgery.

These complications are relatively rare. Have a detailed discussion with your surgeon about the potential risks and complications before undergoing any procedure.

After chordoma surgery, you may undergo additional imaging such as an MRI to determine how much of the tumor was removed. Your next steps will be based on this information, along with your overall health and if the tumor has spread elsewhere in your body.

Radiotherapy after surgery may be recommended, especially if remnants of the tumor are visible. Radiotherapy uses targeted beams of radiation to help eliminate tumor cells that could not be removed during surgery. If the chordoma has spread, or metastasized, chemotherapy may be the next step.

A multidisciplinary team of doctors consisting of neurosurgeons, radiation oncologists, and medical oncologists will be able to guide you through your chordoma treatment process.

• Surgery is the main treatment option for chordomas.
• Skull base chordomas can be surgically removed through the nose or via an incision in the scalp.
• Before deciding to undergo surgery, discuss the benefits and risks of surgery with your surgeon.
• Following surgery, you may require radiotherapy or chemotherapy to eliminate any remaining cancer cells.

• National Cancer Institute: Chordoma
• Chordoma Foundation
• NIH Genetic & Rare Diseases (GARD) Center: Chordoma
• Cancer Net
• American Cancer Society