From the Guidelines
Chordomas are rare, malignant bone tumors that require a multidisciplinary approach for management, with surgery and high-dose 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 (sacral chordomas) 1. Chordomas are aggressive locally, meaning they can invade surrounding tissues and bones, but they rarely spread to distant organs. Symptoms vary depending on tumor location but often include pain, neurological problems, and bowel or bladder dysfunction when located in the sacrum.
Diagnosis and Treatment
Diagnosis typically involves imaging studies like MRI and CT scans, followed by biopsy for confirmation 1. Preoperative core-needle biopsy is recommended, and the biopsy track needs to be included in the surgical resection. Treatment generally requires a multidisciplinary approach, with surgery being the primary intervention to remove as much tumor as possible. Complete removal is challenging due to the tumor's location near critical structures. Following surgery, high-dose radiation therapy, particularly proton beam therapy or carbon ion therapy, is often recommended to target remaining tumor cells.
Prognosis and Recurrence
Unfortunately, chordomas have a high recurrence rate, with loco-regional recurrence affecting >50% of patients treated with macroscopic complete resection with or without RT 1. The overall prognosis depends on tumor location, extent of surgical removal, and whether the tumor has recurred, with 5-year survival rates ranging from 50-70%. 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.
Management of Recurrence
Patients whose tumors recur/progress locally are challenging to control in the long-term and only a minority can be cured. Hence, every effort is needed to maximize the chances for long-term control of tumor with optimal management of the patient at the time of initial treatment. Defining evidence-based best practice to manage this disease state is of utmost importance in order to improve patient outcomes 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, 3.
- They can be encountered anywhere along the axial skeleton, most commonly in the sacral area, skull base, and less commonly in the spine 2.
- 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.
- Conventional chordomas are the most common, characterized by the absence of cartilaginous or additional mesenchymal components 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.
- Symptoms can range from headaches and neck pain to cranial nerve neuropathies and facial numbness, depending on the location of the tumor 4.
Treatment of Chordomas
- There is no standard treatment for chordomas, but surgical resection and high-dose radiation treatment are the mainstays of current treatment 2, 5.
- Aggressive initial therapy improves overall outcome, and patients who relapse locally have a poor prognosis but can be treated with salvage therapy, including radiation and surgery 3.
- Imatinib mesylate, a tyrosine kinase inhibitor, has been found to have antitumor activity in patients with chordoma, particularly those with advanced disease 6.