Characteristics and Classification
Histologically, chordomas are considered low-grade tumors; however, their invasive growth patterns and tendency for recurrence—even after surgery—pose significant clinical challenges.1. Conventional (Classic) Chordoma
The predominant form, characterized by epithelioid cells and slow growth.
2. Chondroid Chordoma
Exhibits features overlapping with chondrosarcoma and is generally associated with a more favorable prognosis.
3. Dedifferentiated Chordoma
The most aggressive subtype, known for rapid progression and invasion into nearby tissues.
A common presentation is chordoma of clivus, which may lead to cranial nerve involvement and base of skull compression. This subtype, known as clival chordoma, requires a multidisciplinary approach due to its proximity to critical neurovascular structures.Common Symptoms of Chordoma
Clinical manifestations depend largely on tumor location. Recognizing early chordoma symptoms is essential for timely intervention. The symptoms of chordoma may include:Head and Neck Pain: Tumors at the skull base cause persistent headaches due to pressure on adjacent structures.
Visual Problems: Involvement of cranial nerves can lead to double vision or partial vision loss.
Swallowing Difficulties: Tumors near the brainstem may disrupt normal swallowing functions.
Limb Weakness: Spinal tumors can compress nerves causing weakness or motor deficits in the arms or legs.
Bowel and Bladder Issues: Sacral tumors often affect spinal cord functions, leading to urinary or fecal incontinence.
Diagnostic Evaluation
Diagnosing chordoma involves a combination of neurological assessment and advanced imaging:Neurological Examination: Focuses on reflexes, muscle strength, coordination, and mental status.
Magnetic Resonance Imaging (MRI): In chordoma radiology: The gold standard for visualizing soft tissue involvement and tumor extent.
Computed Tomography (CT Scan): Provides detailed images of bone structures to assist with surgical planning.
Treatment Strategies
Management depends on tumor characteristics and patient health:1. Surgical Intervention
Surgery remains the cornerstone of treatment, aiming for maximal safe tumor removal.
Microsurgical Resection: Commonly performed for clival chordomas using refined neurosurgical methods.
Endoscopic Endonasal Surgery: A minimally invasive approach accessing skull base tumors through the nasal passages.
2. Radiation Therapy
Chordomas are relatively resistant to traditional radiation; however, radiotherapy is often used after surgery to address residual disease.
Proton Beam Therapy: Delivers precise, high-dose radiation targeting the tumor while sparing healthy tissue, particularly useful for large or surgically inaccessible tumors.
Intensity-Modulated Radiation Therapy (IMRT): Customizes radiation beams to conform to tumor shape, improving targeting accuracy.
3. Targeted Treatments
Certain molecular markers in chordomas make them susceptible to targeted drugs.
Tyrosine Kinase Inhibitors: Agents like imatinib may slow tumor growth by blocking specific growth factor receptors.
4. Chemotherapy
Although generally ineffective against chordomas, chemotherapy may have a role in aggressive, dedifferentiated variants using specialized drug regimens.