Chordoma is a rare, slow-growing, invasive neoplasm that arises from notochordal cells and typically occurs along the midline from the skull base to the sacrum
Pathophysiology
- Chordomas are thought to originate from remnants of the notochord, a structure present during embryonic development
- They are histologically low-grade but highly invasive and often recur locally
Clinical manifestations
- The presentation of chordoma can be insidious, with nonspecific symptoms leading to delayed diagnosis
- Symptoms depend on the tumor's location and may include neurological deficits if the tumor compresses adjacent structures
Risk factors
- Older age, larger tumor size, and distant metastasis are associated with decreased survival
- The ability to achieve a wide en bloc resection at the time of primary surgery is a critical preoperative consideration, as subtotal resections likely complicate later management
Prognosis
- The median survival for chordoma patients is 6 to 7 years
- Prognosis varies substantially among individuals, with a 50% survival rate at 10-year follow-up
- Tumor diameter greater than 5 cm, distant metastasis, and not performing resection are major risk factors that can dramatically decrease the survival time of patients with spinal chordoma
In conclusion, chordoma is a rare, slow-growing, invasive neoplasm with a variable prognosis. The ability to achieve a wide en bloc resection at the time of primary surgery is a critical preoperative consideration, as subtotal resections likely complicate later management