Survival and treatment of cranial and spinal chordomas: a population-based study

Chordomas are rare, slow-growing malignant tumors. Given the paucity of data of the disease, the treatment strategies are disputed. We collected clinical and survival information of patients with chordoma diagnosed between 1975 and 2016 from the Surveillance, Epidemiology, and End Results database. A total of 1797 patients were initially enrolled, including 762 (42.4%) cranial and 1035 (57.6%) spinal chordoma. A total of 1504 patients were further evaluated after screening. In the cranial group, the surgery (gross total resection (GTR): p = 0.001 for overall survival (OS); p = 0.009 for cancer-specific survival (CSS)), tumor extension (distant metastasis: p = 0.001 for OS; p = 0.002 for CSS), and the age (p < 0.001 for OS) were independent prognostic factors for survival. In the spinal group, the age (p = 0.004), location (p < 0.001), GTR (p < 0.001), and tumor extension (distant metastasis, p < 0.001) were independent prognostic factors for OS; the age (p = 0.007), histological type (p < 0.001), GTR (p < 0.001), radiation (p = 0.018), chemotherapy (p = 0.006), and tumor extension (p < 0.001) were independent prognostic factors for CSS. In this large cohort, a significant association was noted between extent of resection and outcome. Even though adjuvant radiation or chemotherapy did not benefit patients with chordoma, the effect on prognosis can be explored in a further study based on our findings.