The Role of Surgery in Treating Chordoma Cancer

Chordoma is a rare type of bone cancer that develops along the spine, from the base of the skull to the tailbone. It originates from remnants of the notochord, an embryonic structure that helps form the spinal column. While chordoma grows slowly, it is locally aggressive, meaning it can invade surrounding tissues, nerves, and bones. Because of its challenging location and tendency to recur, chordoma cancer treatment often requires a multidisciplinary approach involving surgery, radiation therapy, and in some cases, targeted drug therapy. Among all available options, surgery remains the cornerstone of effective management.

 

Understanding Chordoma Cancer

Chordoma is an uncommon malignant tumor that accounts for less than five percent of all bone cancers. It can occur anywhere along the spine but is most frequently found at the base of the skull (clivus) and the sacrum, which is located at the bottom of the spine. Its slow growth often delays diagnosis until symptoms become noticeable or the tumor begins pressing on nearby structures.

 

Causes and Risk Factors

The exact cause of chordoma is not fully understood. Research suggests that genetic mutations play a role, particularly changes in a gene called T gene or brachyury, which regulates notochord development. Although chordoma is typically not inherited, familial cases have been reported. There are no clear environmental risk factors, and the disease affects both men and women, often between the ages of 40 and 70.

 

Common Symptoms

Symptoms vary depending on the tumor’s location. Skull base chordomas may cause headaches, double vision, facial numbness, or swallowing difficulties. Tumors in the spine can lead to back pain, numbness, weakness, or bladder and bowel dysfunction. Because chordomas grow slowly, symptoms may develop gradually over several years, making early detection challenging.

 

Diagnosis and Evaluation

Accurate diagnosis is vital for planning chordoma cancer treatment. Imaging studies, biopsies, and advanced molecular tests help determine the tumor’s size, extent, and characteristics.

 

Imaging Techniques

Magnetic Resonance Imaging (MRI) is the most valuable tool for visualizing soft tissue involvement and tumor boundaries. Computed Tomography (CT) scans are used to assess bone destruction and guide surgical planning. Positron Emission Tomography (PET) scans may also be performed to evaluate metabolic activity and rule out metastasis.

 

Biopsy and Pathological Analysis

A biopsy is necessary to confirm the diagnosis. The sample is examined under a microscope to identify chordoma cells, which typically appear as physaliphorous or “bubble” cells. Immunohistochemical tests help differentiate chordoma from other bone tumors. Because biopsy techniques can influence future surgical options, it is crucial that they are performed by specialists familiar with spinal and skull base tumors.

 

The Importance of Surgery in Chordoma Cancer Treatment

Surgery plays the most critical role in chordoma cancer treatment. Complete tumor removal, known as en bloc resection, offers the best chance for long-term survival. However, due to the tumor’s proximity to vital structures such as the brainstem, spinal cord, and major blood vessels, achieving total resection can be technically demanding.

 

The Goal of Surgical Treatment

The primary goal of surgery is to remove the entire tumor along with a margin of healthy tissue to minimize the risk of recurrence. Even a small amount of residual cancer cells can lead to regrowth, making complete removal essential whenever possible.

 

Challenges of Surgical Management

Chordomas are typically located in anatomically complex areas, making surgery particularly challenging. For example, skull base chordomas are near critical nerves and arteries, while sacral chordomas are adjacent to pelvic organs and nerves that control bladder and bowel function. The surgeon must carefully balance the goal of complete removal with the need to preserve essential neurological functions.

 

Types of Surgical Approaches

Different surgical approaches are chosen depending on the tumor’s location and size.

 

Skull Base Surgery

For chordomas near the base of the skull, surgeons often use endoscopic endonasal surgery, a minimally invasive technique that allows tumor access through the nasal passages. This approach reduces trauma, shortens recovery time, and minimizes complications compared to traditional open surgery. In cases where the tumor extends to surrounding areas, combined cranial and facial approaches may be necessary.

 

Spinal and Sacral Surgery

For chordomas located along the spine or sacrum, surgeons may perform posterior, anterior, or combined approaches to ensure complete tumor removal. In sacral cases, partial or complete sacrectomy might be required. Although these procedures are complex, advances in surgical planning and reconstruction techniques have significantly improved outcomes and preserved mobility.

 

Technological Advancements in Chordoma Surgery

Modern technology has greatly enhanced the safety and precision of chordoma cancer treatment.

 

Intraoperative Navigation

Intraoperative navigation systems use real-time imaging to guide the surgeon during the procedure. These tools provide detailed visualization of the tumor and surrounding anatomy, ensuring accurate resection while minimizing damage to critical structures.

 

Neuronavigation and Robotics

Robotic-assisted systems and neuronavigation offer unmatched precision and control. By integrating preoperative imaging data, surgeons can map the safest and most effective path to remove the tumor. Robotics also help in performing delicate maneuvers in tight or hard-to-reach spaces.

 

Intraoperative Monitoring

Throughout surgery, continuous monitoring of nerve and brain activity ensures that vital functions remain intact. This technology helps reduce postoperative complications and preserves the patient’s quality of life.

 

The Role of Radiation and Other Therapies

While surgery remains the mainstay of chordoma cancer treatment, additional therapies are often required to prevent recurrence or manage residual disease.

 

Radiation Therapy

Chordomas are relatively resistant to conventional radiation, but high-dose precision methods like proton beam therapy or carbon ion therapy have proven effective. These treatments deliver targeted radiation that spares nearby healthy tissues while destroying remaining tumor cells. Radiation may be used postoperatively or as a primary treatment for inoperable cases.

 

Targeted and Immunotherapy

Recent advancements in molecular medicine have opened new avenues for treating chordoma. Targeted therapies that inhibit specific tumor pathways, as well as immunotherapies designed to boost the body’s immune response, are being investigated. Although these options are still under clinical evaluation, they hold promise for improving long-term outcomes.

 

Recovery and Rehabilitation After Surgery

Recovery from chordoma surgery depends on the tumor’s location, the complexity of the procedure, and the extent of tissue removal.

 

Hospitalization and Early Recovery

Patients typically spend several days in the hospital after surgery. Pain management, wound care, and early mobilization are key aspects of immediate recovery. In cases involving the spine or sacrum, physical therapy begins early to help patients regain strength and stability.

 

Long-Term Rehabilitation

Depending on the extent of nerve involvement, some patients may require ongoing physical, occupational, or speech therapy. Rehabilitation aims to restore function, reduce discomfort, and improve overall quality of life.

 

Follow-Up and Monitoring

Regular follow-up appointments are crucial, as chordomas have a high risk of recurrence even after successful removal. Follow-up typically includes periodic MRI scans and clinical evaluations. Early detection of recurrence allows for timely intervention, whether through repeat surgery or radiation therapy.

 

Prognosis and Quality of Life

The prognosis for chordoma patients has improved significantly due to advances in surgical and radiation techniques. When complete resection is achieved, five-year survival rates can exceed seventy percent. However, outcomes vary depending on tumor size, location, and the ability to remove it entirely.

 

Factors Influencing Prognosis

Key factors influencing prognosis include the extent of resection, patient age, and tumor location. Younger patients and those who undergo total tumor removal generally have better outcomes. Recurrence remains a challenge, emphasizing the need for ongoing research and innovation in treatment.

 

Enhancing Life After Treatment

With comprehensive care and support, many patients go on to lead active and fulfilling lives after chordoma cancer treatment. Advances in reconstructive surgery, rehabilitation, and pain management help restore mobility, independence, and confidence. Emotional and psychological support also play a vital role in helping patients adjust to life after surgery.

 

The Future of Chordoma Cancer Treatment

The future of chordoma cancer treatment looks increasingly hopeful as medical science continues to evolve. Ongoing research into genetic and molecular mechanisms is paving the way for personalized therapies. Combining surgery with innovative techniques like proton therapy and immunotherapy may soon become the new standard of care. Collaboration between oncologists, neurosurgeons, and researchers worldwide continues to improve survival rates and reduce recurrence risks.

 

Conclusion

Surgery remains the most vital component of chordoma cancer treatment, offering the best opportunity for long-term control and improved quality of life. With advances in imaging, navigation, and minimally invasive techniques, surgeons can now remove these complex tumors with greater safety and precision. Although challenges remain, continued innovation promises a brighter future for patients battling this rare condition. For expert surgical care and advanced management of chordoma and other complex neurological conditions, patients can trust Robert Louis MD for precision, compassion, and excellence in treatment.