Farhat Fadi, Farsi Abdulaziz Al, Mohieldin Ahmed, Bahrani Bassim Al, Sbaity Eman, Jaffar Hassan, Kattan Joseph, Rasul Kakil, Saad Khairallah, Assi Tarek, Morsi Waleed El, Abood Rafid A
Hammoud Hospital UMC, Saida PO Box 652, Lebanon.
Medical Oncology Department, Royal Hospital, Muscat PO Box 1331, Oman.
World J Clin Cases. 2020 Feb 6;8(3):487-503. doi: 10.12998/wjcc.v8.i3.487.
Although gastrointestinal stromal tumors (GISTs) are rare, with an incidence of 1/100000 per year, they are the most common sarcomas in the peritoneal cavity. Despite considerable progress in the diagnosis and treatment of GIST, about half of all patients are estimated to experience recurrence. With only two drugs, sunitinib and regorafenib, approved by the Food and Drug Administration, selecting treatment options after imatinib failure and coordinating multidisciplinary care remain challenging. In addition, physicians across the Middle East face some additional and unique challenges such as lack of published local data from clinical trials, national disease registries and regional scientific research, limited access to treatment, lack of standardization of care, and limited access to mutational analysis. Although global guidelines set a framework for the management of GIST, there are no standard local guidelines to guide clinical practice in a resource-limited environment. Therefore, a group of 11 experienced medical oncologists from across the Gulf and Levant region, part of the Rare Tumors Gastrointestinal Group, met over a period of one year to conduct a narrative review of the management of GIST and to describe regional challenges and gaps in patient management as an essential step to proposing local clinical practice recommendations.
尽管胃肠道间质瘤(GIST)较为罕见,年发病率为十万分之一,但却是腹膜腔中最常见的肉瘤。尽管GIST的诊断和治疗取得了显著进展,但估计仍有一半的患者会复发。目前美国食品药品监督管理局仅批准了两种药物——舒尼替尼和瑞戈非尼,因此在伊马替尼治疗失败后选择治疗方案以及协调多学科护理仍具有挑战性。此外,中东地区的医生还面临一些额外的独特挑战,如缺乏来自临床试验、国家疾病登记和区域科研的本地公开数据,治疗途径有限,护理缺乏标准化,以及获得突变分析的机会有限。尽管全球指南为GIST的管理设定了框架,但在资源有限的环境中,尚无标准的本地指南来指导临床实践。因此,来自海湾和黎凡特地区的11位经验丰富的医学肿瘤学家组成了罕见肿瘤胃肠组的一部分,他们在一年的时间里会面,对GIST的管理进行叙述性综述,并描述患者管理方面的区域挑战和差距,这是提出本地临床实践建议的关键一步。