医学
临床试验
疾病
免疫疗法
化疗
重症监护医学
透明细胞肉瘤
全身疗法
肉瘤
肿瘤科
内科学
病理
癌症
乳腺癌
作者
Jasmien Cornillie,Thomas Van Cann,Agnieszka Wozniak,Daphne Hompes,Patrick Schöffski
标识
DOI:10.1080/14737140.2016.1197122
摘要
Clear cell sarcoma (CCS) is an aggressive tumor, typically developing in tendons or aponeuroses. The outcome of this orphan disease is poor, with 5-year and 10-year survival rates of localized CCS around 60-70% and 40-50%. Once the disease has metastasized, it is usually fatal due to its chemotherapy-resistant nature. Systemic treatment options are poorly standardized and the use of chemotherapy is based on weak scientific evidence.In this review, we systematically discuss the current scientific evidence for the systemic treatment of CCS, including tyrosine kinase inhibitors, immunotherapy and MET inhibitors. Expert commentary: Recent insights in the biology of CCS have identified new potential therapeutic targets, which should be tested in prospective clinical trials. Whenever possible, patients with metastatic CCS should be included in clinical trials with good biological rationale. Innovative trial methodology and new regulatory mechanisms are required to provide patients with uncommon cancers with active drugs.
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