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单机构中的胃肠道间质瘤:与其他胃肠道恶性肿瘤有关联吗?

Gastrointestinal stromal tumours in a single institute: is there an association to other gastrointestinal malignancies?

作者信息

Kalmár Katalin, Tornóczky Tamás, Pótó László, Illényi László, Kalmár Nagy Károly, Kassai Miklós, Kelemen Dezso, Horváth Ors Péter

机构信息

Department of Surgery, University of Pécs, Hungary.

出版信息

Magy Seb. 2004 Oct;57(5):251-6.

Abstract

Our knowledge of Gastrointestinal Stromal Tumours (GIST) has been broadened in the last few years by the discovery of the key function of c-kit tyrosine kinase mutation in their pathogenesis. A single institution database is presented and a hypothesis is examined regarding probable connection between GIST and other gastrointestinal malignancies. Between 2000 and 2004 20 patients were admitted to our department with GIS tumour. With the addition of three cases detected between 1991 and 2000, and proved to be GIST retrospectively, 23 cases are followed. Mean age was 64.52 years, male to female ratio 11:12. Ten tumours originated from the stomach, ten from small bowel, one from rectum and two from mesenterium. All patients underwent surgery, 16 operations were completed with R0 radicality, one R1 and four R2 resections were performed. Follow-up ranged from 1 to 157 months. Five out of 23 patients died (21.7%), six patients live with metastatic disease, twelve are disease free. Amongst patient-, tumour- and treatment-related factors the prognostic significance of Fletcher's risk and radicality of resection was demonstrated (p<0.05). Significantly more synchronous or metachronous gastrointestinal malignancies were found is this population of GIST patients, when compared to the prevalence of malignancies in normal Hungarian population (p<0.001).

摘要

在过去几年中,随着c-kit酪氨酸激酶突变在胃肠道间质瘤(GIST)发病机制中的关键作用被发现,我们对GIST的认识得到了拓宽。本文展示了一个单一机构的数据库,并检验了关于GIST与其他胃肠道恶性肿瘤之间可能联系的假说。2000年至2004年间,有20例GIST患者入住我们科室。加上1991年至2000年间检测到的3例病例(这些病例后来被回顾性证实为GIST),共有23例病例被随访。平均年龄为64.52岁,男女比例为11:12。10例肿瘤起源于胃,10例起源于小肠,1例起源于直肠,2例起源于肠系膜。所有患者均接受了手术,16例手术实现了R0根治性切除,1例为R1切除,4例为R2切除。随访时间为1至157个月。23例患者中有5例死亡(21.7%),6例患者伴有转移性疾病存活,12例无疾病。在患者、肿瘤和治疗相关因素中,Fletcher风险和切除根治性的预后意义得到了证实(p<0.05)。与匈牙利正常人群中恶性肿瘤的患病率相比,在这群GIST患者中发现的同时性或异时性胃肠道恶性肿瘤明显更多(p<0.001)。

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