• Cas Vanderheijden, Thomas Vaessen, Youssef Yakkioui, Yasin Temel, Govert Hoogland, and Koos Hovinga.
• Department of Neurosurgery, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Neuroscience, School for Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands. Electronic address: c.vanderheijden@maastrichtuniversity.nl.
• World Neurosurg. 2021 Dec 1; 156: 125-132.

BackgroundA chordoma is a slow-growing, invasive neoplasm in the neuraxis that is thought to arise from notochordal cells. At 10-year follow-up, the average survival rate is 50%, though individual prognosis varies substantially. We aimed to provide a comprehensive overview of the genes and proteins expressed in these tumors and their prognostic value to facilitate prognostication for patients with chordoma.MethodsA systematic search of clinical studies that investigated expressed factors related to chordoma survival was performed in PubMed. Data extracted included RNA and protein expression data and prognostic value (in terms of overall survival, progression-free survival, disease-free survival, and recurrence-free survival) from univariate and multivariate analyses.ResultsThis review included 78 original studies that collectively evaluated 134 expressed factors. Of these molecular factors, 96 by univariate analysis and 32 by multivariate analysis had a predictive value for patient survival. Of the molecular factors studied in multivariate analyses, 26 factors had a negative effect while 6 had a positive effect on patient survival.ConclusionsIdentification of molecular factors that are associated with survival contributes to better prognostication of patients with chordoma. Given the rarity of chordoma, often only univariate analyses can be performed. Robust multivariate analyses are scarcer but provide independently significant prognostic factors. The data presented in this review can aid in prognostication for the individual patient and facilitate the development of targeted therapies.Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

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