Chordomas of the skull base, mobile spine, and sacrum: an epidemiologic investigation of presentation, treatment, and survival.

Chordomas of the skull base, mobile spine, and sacrum: an epidemiologic investigation of presentation, treatment, and survival.

World Neurosurg. 2018 Feb 24;:

Authors: Zuckerman SL, Bilsky MH, Laufer I

Abstract
BACKGROUND: Chordomas are rare, primary bone tumors that arise from the axial skeleton. Our objective was to analyze trends in radiation and surgery over time and determine location-based survival predictors for chordomas of the skull base, mobile spine, and sacrum.
METHODS: A retrospective cohort study of the Surveillance Epidemiology and End Results (SEER) database from 1973-2013 was conducted. All patients had histologically confirmed chordomas. The principal outcome measure was overall survival (OS).
RESULTS: The cohort included 1,616 patients: skull base (664), mobile spine (444), and sacrum (508). Skull base tumors presented earliest in life (47.4 years) and sacral tumors presented latest (62.7 years). Rates of radiation remained stable for skull base and mobile spine tumors but declined for sacral tumors (p=0.006). Rates of surgical resection remained stable for skull base and sacral tumors but declined for mobile spine tumors (p=0.046). Skull base chordomas had the longest median survival (162 months) compared to mobile spine (94 months) and sacral tumors (87 months). Being married was independently associated with improved OS for skull base tumors (HR0.73, 95%CI 0.53-0.99, p=0.044). Surgical resection was independently associated with improved OS for sacral chordomas (HR0.48, 95%CI 0.34-0.69, p<0.001).
CONCLUSIONS: Surgical resection for mobile spine chordomas and radiation for sacral chordomas decreased. Patients with skull base tumors survived longer than patients with mobile spine and sacral chordomas, and surgical resection was associated with improved survival in sacral chordomas only. Understanding the behavior of these tumors can help cranial and spinal surgeons improve treatment in this patient population.

PMID: 29486315 [PubMed - as supplied by publisher]

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