Cervical chordoma is a rare malignant bone tumor affecting the bones of the neck, specifically the cervical spine. This type of tumor originates from remnants of the notochord, a flexible rod-like structure present during embryonic development that typically disappears before birth. As a type of sarcoma, it develops in connective tissues.
What is Cervical Chordoma?
Chordoma is a rare malignant tumor that can arise anywhere along the central neural axis, with approximately 30% of cases developing in the vertebrae of the mobile spine, including the cervical spine. While chordomas are most commonly found in the sacrococcygeal region (base of the spine) and the base of the skull, cervical chordomas specifically occur in the seven bones that make up the neck. These tumors are known for their slow growth, often developing over years without immediate symptoms.
Despite their slow growth, cervical chordomas are locally aggressive, meaning they can gradually invade and damage surrounding bone and tissues. Their proximity to delicate structures like the brainstem, spinal cord, and major nerves and arteries in the neck makes them particularly challenging to treat. It is important to distinguish cervical chordoma from more common spinal tumors, as its unique origin and characteristics require specialized management.
Identifying Symptoms
Cervical chordoma symptoms often develop gradually, progressing as the tumor grows and exerts pressure on nearby structures. Persistent neck pain is a common manifestation, which may be accompanied by stiffness or reduced range of motion in the neck. This pain can radiate to the shoulders or arms, depending on the tumor’s location.
As the tumor enlarges, it can compress the spinal cord or nerves, leading to neurological symptoms. These may include weakness, numbness, or tingling sensations in the arms and hands. If the tumor presses on the esophagus or other structures in the throat, individuals might experience difficulty swallowing (dysphagia) or changes in their voice, such as hoarseness.
Diagnosis Process
Diagnosing cervical chordoma begins with a thorough physical and neurological examination. Healthcare providers assess for signs of nerve compression, muscle weakness, reflexes, and sensation.
Imaging studies are then employed to visualize the tumor and determine its extent. Magnetic Resonance Imaging (MRI) provides detailed images of soft tissues and the spinal cord. Computed Tomography (CT) scans offer clearer views of bone involvement, while Positron Emission Tomography (PET) scans may be used to identify potential spread of the tumor.
A biopsy is the most definitive step in confirming a cervical chordoma diagnosis. This involves taking a tissue sample from the tumor, which a pathologist examines under a microscope. The biopsy helps to differentiate chordoma from other spinal conditions or tumors. A multidisciplinary team reviews these findings to understand the tumor comprehensively.
Treatment Options
The primary goal for treating cervical chordoma is complete surgical removal of the tumor, also known as en bloc resection. This procedure can be highly complex due to the tumor’s close proximity to the spinal cord, brainstem, and major blood vessels in the neck. Specialized surgical teams, often comprising neurosurgeons and orthopedic spine surgeons, collaborate to maximize tumor removal while preserving neurological function.
If complete removal is not possible or microscopic tumor cells remain, high-dose, highly conformal radiation therapy is used as an adjuvant treatment. Techniques like proton beam therapy or carbon ion therapy deliver precise radiation doses to the tumor while minimizing exposure to surrounding healthy tissues. Radiation can also be a primary treatment option if surgery is not feasible.
Newer treatment approaches, such as targeted therapies, are emerging, particularly for advanced or recurrent cases. These therapies focus on specific genetic mutations found within the tumor cells, such as those involving the BRAF gene. While still evolving, these systemic treatments offer additional options to manage the disease. The management of cervical chordoma consistently involves a multidisciplinary team, including neurosurgeons, radiation oncologists, and medical oncologists, who work together to create an individualized treatment plan.
Prognosis and Long-Term Outlook
The prognosis for individuals with cervical chordoma is influenced by several factors, including the extent of tumor removal achieved during surgery and the tumor’s specific location. The tumor’s response to radiation therapy also plays a significant role in the long-term outlook. While chordomas are slow-growing, they have a tendency to recur locally in the same area after treatment.
Regular follow-up imaging, such as MRI scans, is therefore important to monitor for any signs of recurrence. This ongoing surveillance allows for early detection and intervention if the tumor begins to grow again. Patients are typically followed closely for many years after treatment to manage potential late effects and ensure the best possible quality of life.