Incidence and relative survival of chordomas

Abstract

BACKGROUND: Chordomas are rare bone tumors arising from remnants of the embryonic notochord. METHODS: Data for this study were obtained from the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute to calculate the incidence, relative survival (RS), and standardized mortality ratio (SMR) of patients diagnosed with intracranial and extracranial chordomas and to assess the effects of age and sex on this disease. RESULTS: The overall incidence of extracranial and intracranial chordomas was 8.4 per 10 million population. The median overall survival of patients with chordoma patients was 7.7 years. The median survival was 7.7 years for male patients and 7.8 years for female patients. Younger patients (aged <40 years) survived longer compared with older patients (10-year RS, 68% vs 43%). The estimated age-standardized 5-year, 10-year, and 20-year RS rates was 72%, 48%, and 31%, respectively. The SMR in the overall cohort was 4.6 (95% confidence interval, 4.22-5.0) or 21.0 (95% confidence interval, 16.6-27.2) in young adult patients and 3.0 (95% confidence interval, 2.6-3.4) in elderly patients. CONCLUSIONS: The elderly had a more aggressive form of this disease; and, other than the incidence, sex did not influence outcome in this disease. The study of chordomas presents a good case for the contribution that the SMR can have on measuring the impact of a disease on a population of patients. Although the younger population has better survival rates, the impact (SMR) in the younger age groups is much higher than in older populations.

Incidence Patterns of Primary Bone Cancer in Taiwan (2003–2010): A Population-Based Study

“…The overall age-specific incidence patterns reported in this study were consistent with those reported in Western countries,2,3,12–20 showing that OS and ES are more likely to be diagnosed in youth, whereas CS and GCT occur more frequently in middle-aged and elderly individuals, and chordomas and ameloblastomas predominantly affect elderly patients. However, more details remain to be clarified.…”

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011)

“…Primary chordomas are slow-growing, locally destructive, rare bone tumors arising from notochord tissue along the cranial-spinal axis [6,7,8,9]. Proximity to the brainstem, cranial nerves, and major intracranial vessels make this histologically low-grade neoplasm clinically malignant [7].…”

Primary Intestinal and Vertebral Chordomas in Laboratory Zebrafish (Danio rerio)

“…The overall incidence is reported to be 8.4 per ten million, with a higher incidence in males and individuals older than 40 years. 11,16 Tumors are reported to arise in the cranium (frequently clival, 32%), spine (33%) and sacrum (29%). 11,16 Chordomas are typically lobulated with fibrous trabeculae, and the presence of numerous vacuolated (physaliphorous) cells is characteristic.…”

“…11,16 Tumors are reported to arise in the cranium (frequently clival, 32%), spine (33%) and sacrum (29%). 11,16 Chordomas are typically lobulated with fibrous trabeculae, and the presence of numerous vacuolated (physaliphorous) cells is characteristic. 12 Although they infrequently metastasize, tumors are typically locally invasive with frequent recurrence, often owing to the difficulty of complete resection.…”

“…12 Although they infrequently metastasize, tumors are typically locally invasive with frequent recurrence, often owing to the difficulty of complete resection. 16 Extra-axial skeletal chordomas are rare in people; extra-skeletal soft tissue chordomas are exceptionally rare, with less than twenty reported cases. 9 …”