Σφακιανάκης Αλέξανδρος
ΩτοΡινοΛαρυγγολόγος
Αναπαύσεως 5 Άγιος Νικόλαος
Κρήτη 72100
00302841026182
00306932607174
alsfakia@gmail.com

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Δευτέρα 14 Νοεμβρίου 2016

Clinical features and prognostic factors of children and adolescent patients with clival chordomas.

Clinical features and prognostic factors of children and adolescent patients with clival chordomas.

World Neurosurg. 2016 Nov 9;:

Authors: Zhai Y, Bai J, Gao H, Wang S, Li M, Gui S, Li C, Zhang Y

Abstract
OBJECTIVE: Chordomas in children and adolescents are extremely uncommon, and minimal published information is available. Therefore, our aim was to research clinical features and prognostic factors associated with clival chordomas in younger patients.
METHODS: The study included 25 patients (10 males and 15 females). The average follow-up was 42.95 months (range: 2 to 108 months). Kaplan-Meier analysis was used to determine overall survival (OS) and progression-free survival (PFS). Pearson correlation was used to perform correlation analysis. Immunohistochemistry was used to detect the expression levels of Ki67, cytokeratin 8, platelet-derived growth factor receptor-β (PDGFR-β), and Brachyury.
RESULTS: The average age (± SD) of patients was 14.44 (3.44) years. Total resection (TR) and non-total resection (NTR) was achieved in 5 cases and 20 cases, respectively. The average OS of patients with a Karnofsky performance score (KPS) ≥90 or those in the TR group was significantly longer compared with that of patients with KPS<90 group (p=0.041) or those in the NTR group (p=0.0497). Patients with lower Ki67 expression levels had longer OS (p=0.0207). The PFS of patients with lower CK8 expression levels was significantly longer compared with those with higher levels (p=0.008). The expression levels of PDGFR-β was significantly correlated with those of Brachyury (p=0.016).
CONCLUSION: In children and adolescent patients with clival chordomas, higher pre-operative KPS, total resection, and lower levels of Ki67 and CK8 expression were favorable prognostic factors. PDGFR-β might play a role in tumorigenesis and epithelial-mesenchymal transition of clival chordomas.

PMID: 27838423 [PubMed - as supplied by publisher]



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