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十二指肠胃肠道间质瘤患者的临床病理特征、手术及生存结果

Clinicopathological Characteristics, Surgery and Survival Outcomes of Patients with Duodenal Gastrointestinal Stromal Tumors.

作者信息

Sugase Takahito, Takahashi Tsuyoshi, Nakajima Kiyokazu, Hirota Seiichi, Masuzawa Toru, Nishida Toshirou, Kimura Yutaka, Miyazaki Yasuhiro, Makino Tomoki, Kurokawa Yukinori, Yamasaki Makoto, Takiguchi Shuji, Mori Masaki, Doki Yuichiro

机构信息

Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Suita, Japan.

出版信息

Digestion. 2016;94(1):30-6. doi: 10.1159/000447665. Epub 2016 Jul 13.

Abstract

BACKGROUND

Duodenal gastrointestinal stromal tumors (GISTs) are a rare subset of GISTs (3-5%), and their clinicopathological features have not been fully described. The purpose of this retrospective study was to examine these characteristics and compare the operative procedures.

METHODS

Twenty-five patients with duodenal GIST underwent complete resection, local resection (LR) or pancreaticoduodenectomy (PD) from 1990 to 2014 at our 2 hospitals. We analyzed patient characteristics, treatments, histological examinations, postoperative complications and survival outcomes.

RESULTS

Twelve patients (48%) with no symptoms were incidentally diagnosed for unrelated reasons. Sixteen patients (64%) had c-kit mutations while 6 (24%) were wild-type, including 4 with a history of neurofibromatosis type 1. Comparing LR (n = 16) and PD (n = 9), the recurrence-free survival rate was significantly worse for PD. On multivariate analysis, however, tumor size was an independent and significant prognostic factor, but not operative procedure. There was no body weight change with LR, but body weight decreased by 7% with PD.

CONCLUSION

Duodenal GISTs had different characteristic genetic mutations compared to other GISTs. LR for duodenal GISTs appears to be oncologically and nutritionally feasible.

摘要

背景

十二指肠胃肠道间质瘤(GISTs)是GISTs中较为罕见的一个亚型(占3 - 5%),其临床病理特征尚未得到充分描述。本回顾性研究的目的是探讨这些特征并比较手术方式。

方法

1990年至2014年期间,我们两家医院的25例十二指肠GIST患者接受了根治性切除、局部切除(LR)或胰十二指肠切除术(PD)。我们分析了患者特征、治疗方法、组织学检查、术后并发症及生存结果。

结果

12例(48%)无症状患者因无关原因被偶然诊断。16例(64%)患者存在c - kit基因突变,6例(24%)为野生型,其中4例有1型神经纤维瘤病病史。比较LR组(n = 16)和PD组(n = 9),PD组的无复发生存率明显更差。然而,多因素分析显示,肿瘤大小是一个独立且显著的预后因素,而非手术方式。LR组患者体重无变化,但PD组患者体重下降了7%。

结论

与其他GISTs相比,十二指肠GISTs具有不同特征的基因突变。十二指肠GISTs的LR在肿瘤学和营养方面似乎是可行的。

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