From the Guidelines
Chordomas are rare, malignant bone tumors that require a multidisciplinary approach for management, with surgery and radiation therapy being the primary interventions to achieve long-term control and improve quality of life. These tumors typically occur along the spine, most commonly at the base of the skull (clival chordomas) or in the sacrum (the lowest part of the spine) [ 1 ]. Chordomas are aggressive locally, meaning they can invade surrounding tissues and bones, but they rarely spread to distant organs [ 1 ]. Symptoms vary depending on tumor location but often include pain, neurological problems, and bowel or bladder dysfunction.
Diagnosis and Treatment
Diagnosis typically involves imaging studies like MRI and CT scans, followed by a biopsy for confirmation [ 1 ].
- MRI is the best modality for local staging
- CT scan should be used in the case of diagnostic doubt
- Chordoma should be differentiated from benign notochordal lesions and, if radiological appearance is typical for these, biopsy is not recommended unless the lesion changes with time
- Preoperative core-needle biopsy is recommended
- The biopsy track needs to be included in the surgical resection Treatment usually requires a multidisciplinary approach, with surgery being the primary intervention to remove as much tumor as possible [ 1 ]. Complete removal is challenging due to the tumor's location near critical structures. Radiation therapy, particularly proton beam therapy or carbon ion therapy, is often used after surgery to target remaining tumor cells.
Prognosis and Recurrence
Unfortunately, chordomas have a high recurrence rate, and patients require long-term monitoring [ 1 ]. The overall prognosis depends on factors like tumor location, extent of surgical removal, and whether the tumor has invaded surrounding structures. Major determinants of local control in primary chordomas at all sites include tumor size, extent of resection, quality of surgery, quality of RT, and patient age [ 1 ]. Newer targeted therapies are being investigated for cases that don't respond to standard treatments. Long-term disease control and good quality of life may still be possible for some patients with optimal management [ 1 ].
From the Research
Definition and Characteristics of Chordomas
- Chordomas are rare, slow-growing tumors of the axial skeleton, which derive from the remnants of the fetal notochord 2.
- They can be encountered anywhere along the axial skeleton, most commonly in the sacral area, skull base, and less commonly in the spine 2, 3.
- Chordomas have a benign histopathology but exhibit malignant clinical behavior with invasive, destructive, and metastatic potential 2.
Pathological Subtypes of Chordomas
- There are 3 pathological subtypes of chordomas: classic, chondroid, and dedifferentiated chordomas 2, 3.
- Chondroid chordomas contain both chordomatous and chondromatous features and have a predilection for the spheno-occipital region of the skull base 3.
- Dedifferentiation or sarcomatous transformation occurs in 2%-8% of chordomas 3.
Diagnosis and Symptoms of Chordomas
- Chordomas most commonly present with headaches and diplopia and can be readily diagnosed by current neuroradiological methods 2.
- When developing intracranially, symptoms can range from headaches and neck pain to cranial nerve neuropathies and facial numbness 4.
- Radiological studies can reveal a large heterogeneous mass in the spheno-occipital region with clivus destruction 4.
Treatment and Prognosis of Chordomas
- Surgical resection and high-dose radiation treatment are the mainstays of current treatment 2, 5.
- The outcome is dictated primarily by the intrinsic biology of the tumor, and treatment seems only to have a secondary impact 2.
- A significant percentage of skull base chordomas recur despite treatment, and the recurrence rate ranges from 25% to 60% for cervical chordomas and from 18% to 89% for sacrococcygeal chordomas 6.
- Adjuvant radiation therapy increases the continuous disease-free survival and the local recurrence-free survival 6, 5.