Spinal column chordoma: prognostic significance of clinical variables and T (brachyury) gene SNP rs2305089 for local recurrence and overall survival.
Neuro Oncol
; 19(3): 405-413, 2017 03 01.
Article
de En
| MEDLINE
| ID: mdl-27663388
ABSTRACT
Background:
Chordomas are rare, locally aggressive bony tumors associated with poor outcomes. Recently, the single nucleotide polymorphism (SNP) rs2305089 in the T (brachyury) gene was strongly associated with sporadic chordoma development, but its clinical utility is undetermined.Methods:
In 333 patients with spinal chordomas, we identified prognostic factors for local recurrence-free survival (LRFS) and overall survival and assessed the prognostic significance of the rs2305089 SNP.Results:
The median LRFS was 5.2 years from the time of surgery (95% CI 3.8-6.0); greater tumor volume (≥100cm3) (hazard ratio [HR] = 1.99, 95% CI 1.26-3.15, P = .003) and Enneking inappropriate resections (HR = 2.35, 95% CI 1.37-4.03, P = .002) were independent predictors of LRFS. The median overall survival was 7.0 years (95% CI 5.8-8.4), and was associated with older age at surgery (HR = 1.11 per 5-year increase, 95% CI 1.02-1.21, P = .012) and previous surgical resection (HR = 1.73, 95% CI 1.03-2.89, P = .038). One hundred two of 109 patients (93.6%) with available pathologic specimens harbored the A variant at rs2305089; these patients had significantly improved survival compared with those lacking the variant (P = .001), but there was no association between SNP status and LRFS (P = .876).Conclusions:
The ability to achieve a wide en bloc resection at the time of the primary surgery is a critical preoperative consideration, as subtotal resections likely complicate later management. This is the first time the rs2305089 SNP has been implicated in the prognosis of individuals with chordoma, suggesting that screening all patients may be instructive for risk stratification.Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Tumeurs du rachis
/
Chordome
/
Marqueurs biologiques tumoraux
/
Protéines à domaine boîte-T
/
Polymorphisme de nucléotide simple
/
Protéines foetales
Type d'étude:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limites:
Female
/
Humans
/
Male
/
Middle aged
Langue:
En
Journal:
Neuro Oncol
Sujet du journal:
NEOPLASIAS
/
NEUROLOGIA
Année:
2017
Type de document:
Article
Pays d'affiliation:
États-Unis d'Amérique