Skull base chordomas review of current treatment paradigms  

作　　者：Dan Yaniv Ethan Soudry Yulia Strenov Marc A.Cohen Aviram Mizrachi 

机构地区：[1]Department of Otolaryngology-Head and Neck Surgery,Rabin Medical Center,Petah Tikva and Sackler Faculty of Medicine,Tel Aviv University,Tel Aviv,Israel [2]Department of Pathology,Rabin Medical Center,Petah Tikva and Sackler Faculty of Medicine,Tel Aviv University,Tel Aviv,Israel [3]Head and Neck Service,Department of Surgery,Memorial Sloan-Kettering Cancer Center,New York,NY,USA

出　　处：《World Journal of Otorhinolaryngology-Head and Neck Surgery》2020年第2期125-131,共7页世界耳鼻咽喉头颈外科杂志（英文）

基　　金：This research was funded in part through the NIH/NCI Cancer Center Support Grant,P30 CA008748.

摘　　要：Background:Chordomas are locally invasive neoplasms,arising from notochordal remnants and can appear anywhere along the axial skeleton.Local recurrences are common,and distant metastases may occur years after the initial presentation.Methods:Literature review of current treatment strategies for chordomas of the skull base.Results:Surgery is the mainstay of treatment and complete resection has paramount importance for prognosis.When complete resection is not achieved recurrent disease is common.The anatomical complexity of the skull base makes resection complex.Endonasal endoscopic approaches to the clivus has become increasingly favored in recent years although addressing reconstruction of the skull base to prevent CSF leak may be challenging.Evidence suggests that radiotherapy should not be considered as a primary single modality when trying to achieve cure of the disease.Nonetheless,immediate post-operative radiotherapy improves survival.Many strategies have been suggested to preserve sensitive vital structures in the skull base during treatment but as for survival there is no evidence of advantage when comparing adjuvant therapy with photon radiotherapy,gamma knife surgery,proton beam therapy,and carbon ion radiation therapy.There is no evidence to support cytotoxic chemotherapy in the treatment of chordomas but targeted therapies have started to show promise.Several optional molecular targets exist.Brachyury is overexpressed in 95%of chordomas but not in other mesenchymal neoplasms.However,its precise role in chordoma pathogenesis is currently unclear,and its cellular location in the nucleus makes it difficult to target.The inhibition of brachyury in chordoma cell lines induces growth arrest and apoptosis.This does not have clinical application to date.There are retrospective results with different molecular targeted therapies for advanced chordomas with some effectiveness.Conclusion:Despite improvements made in the past 10 years in our knowledge of chordoma biology,available therapies still offer a limited b

关 键 词：Skull Base CHORDOMA SURGERY REVIEW Targeted therapy 

分 类 号：R739.41[医药卫生—肿瘤]