Chordoma is a rare type of cancer that originates in the bones. It arises from remnants of the notochord, a flexible rod-like structure present during embryonic development that later forms part of the spine. Chordoma is a rare primary bone cancer, accounting for a small percentage of all such cancers.
What is Spinal Chordoma
Spinal chordoma specifically develops from notochordal cells that persist along the vertebral column after birth. These tumors most frequently occur at the ends of the spine, with the sacrum (the bone at the base of the spine) being the most common site. They can also appear in the mobile spine, including the cervical (neck), thoracic (mid-back), or lumbar (lower back) regions.
Unlike chordomas that arise at the skull base, spinal chordomas present unique challenges due to their proximity to the spinal cord and nerves. This tumor grows slowly over months or even years, but it is considered locally aggressive, invading surrounding bone and soft tissues. Its slow growth can sometimes delay diagnosis, as symptoms may not become apparent until the tumor has reached a considerable size.
Recognizing the Symptoms
Symptoms associated with spinal chordoma develop gradually and can be non-specific. Patients may experience persistent pain localized to the area of the tumor, such as lower back pain for sacral tumors or neck pain for cervical spine involvement. This pain worsens over time and may not be relieved by rest or common pain relievers.
As the tumor grows, it can compress the spinal cord or adjacent nerves, leading to various neurological deficits. These can include weakness, numbness, or tingling sensations in the limbs, depending on which nerves are affected. For tumors in the lower spine, individuals might experience changes in bowel or bladder function due to nerve compression. Radiculopathy, characterized by pain, numbness, or weakness radiating along the path of a nerve, is also a common symptom when nerve roots are impinged.
How Spinal Chordoma is Diagnosed
Diagnosis begins with a thorough medical history and a comprehensive physical examination. A physician will inquire about the nature, duration, and progression of symptoms, along with any neurological changes. The physical exam assesses muscle strength, reflexes, and sensation to identify areas of potential nerve compression.
Imaging studies play a central role in identifying the presence and extent of the tumor. Magnetic Resonance Imaging (MRI) is the preferred imaging modality, providing detailed images of soft tissues, the spinal cord, and nerves to delineate the tumor’s size and its relationship to surrounding structures. Computed Tomography (CT) scans are also used, particularly to assess bone destruction and provide a clearer view of the bony anatomy. While imaging can strongly suggest a chordoma, a definitive diagnosis requires a biopsy. During a biopsy, a small tissue sample is extracted from the tumor, often guided by imaging, and then examined under a microscope by a pathologist. Due to the rarity of chordoma, specialized pathology review by experts is sought to confirm the diagnosis and distinguish it from other bone lesions.
Treatment Approaches
Treating spinal chordoma involves a multidisciplinary approach. Surgical resection is the primary treatment, aiming for maximal safe tumor removal. The goal of surgery is to remove as much of the tumor as possible while preserving neurological function, such as motor skills and sensation. This requires complex procedures, especially when the tumor is close to the spinal cord or major nerves.
Achieving clear surgical margins, where no tumor cells are left behind at the edge of the removed tissue, is a significant challenge due to the tumor’s infiltrative nature and proximity to delicate structures. Even with extensive surgery, microscopic tumor cells may remain, which necessitates further treatment. Radiation therapy follows surgery to target any residual tumor cells and reduce recurrence risk. High-dose, highly conformal radiation, such as proton beam therapy or carbon ion therapy, is preferred for chordoma. These advanced techniques deliver a precise dose of radiation to the tumor while minimizing exposure to surrounding healthy tissues, which is particularly important given the spinal cord’s sensitivity.
In cases where surgery is not feasible due to the tumor’s location or extent, radiation therapy may be used as the primary treatment. Systemic therapies, including targeted therapies and conventional chemotherapy, have a more limited role in the treatment of chordoma compared to surgery and radiation. Targeted therapies, which focus on specific molecular pathways involved in cancer growth, are being investigated and may be considered for advanced or recurrent cases. However, chordoma does not respond well to traditional chemotherapy.
Life After Diagnosis
Life after a spinal chordoma diagnosis involves ongoing management and monitoring due to the tumor’s potential for local recurrence. Regular follow-up imaging with MRI scans is performed at scheduled intervals to detect any tumor regrowth as early as possible. Scan frequency may vary but begins every few months in the initial years after treatment, gradually decreasing if the tumor remains stable.
Rehabilitation plays an important role in managing any functional deficits that may arise from the tumor itself or from treatment. Physical therapy can help individuals regain strength, mobility, and balance, while occupational therapy focuses on adapting daily activities to improve independence. Some individuals may require pain management strategies to address persistent discomfort. Supportive care, which includes psychological support and nutritional guidance, also contributes to overall well-being.