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小胃肠道间质瘤(<2厘米)的癌症生物学:恶性风险是什么?

Cancer biology of small gastrointestinal stromal tumors (<2 cm): What is the risk of malignancy?

作者信息

Søreide K

机构信息

Department of Gastrointestinal Surgery, Stavanger University Hospital, PO Box 8100, N-4068 Stavanger, Norway; Gastrointestinal Translational Research Unit, Laboratory of Molecular Medicine, Stavanger University Hospital, Stavanger, Norway; Department of Clinical Medicine, University of Bergen, Bergen, Norway.

出版信息

Eur J Surg Oncol. 2017 Jul;43(7):1344-1349. doi: 10.1016/j.ejso.2017.01.240. Epub 2017 Feb 9.

DOI:10.1016/j.ejso.2017.01.240
PMID:28222971
Abstract

INTRODUCTION

The identification of small (<2 cm) gastrointestinal stromal tumors (GISTs) is increasingly recognized. The malignancy potential is not absent. However, data on risk is scarce. The aim was to review the existing data on perceived risk of small GISTs in population-based studies.

METHODS

A combined review of small GISTs (<2 cm) in a population-based dataset compared with a systematic review of available population-based studies.

RESULTS

About one in every four (27%) patient has a small GIST, of which 79% were incidental in presentation, and all had a low-risk mitoses index (<5/hpf). The small GISTs had C-KIT mutations in exon 11 or 9 (66%), none had mutation in PDGFRA, and 33% were non-KIT/PDGFRA. The rate of small GISTs increased for each advancing age group. None of the small GISTs had a recurrence nor were there any GIST-specific deaths during follow-up. A "small GIST" rate at one-fourth of all GISTs reported was corroborated in other population-based studies from Norway, Iceland and Korea. Common to studies reporting mitosis index were an almost universal finding of very low-risk in small GISTs, with very few recurrences and an excellent long-term survival reported to 95-100% in several series.

DISCUSSION

One in every four GIST diagnosed is a small GIST (<2 cm). Small GISTs are increasingly found with advanced age and have an overall excellent prognosis. Defining true risk-features for proper surveillance and treatment strategy is needed in order to avoid over- and under-treatment.

摘要

引言

小(<2厘米)胃肠道间质瘤(GIST)的识别越来越受到重视。其恶性潜能并非不存在。然而,关于风险的数据却很稀少。目的是回顾基于人群研究中有关小GIST感知风险的现有数据。

方法

对基于人群的数据集中的小GIST(<2厘米)进行综合回顾,并与现有基于人群研究的系统评价进行比较。

结果

约每四名患者中就有一名(27%)患有小GIST,其中79%在表现上为偶然发现,且所有患者的有丝分裂指数均为低风险(<5/高倍视野)。小GIST在第11或9外显子中有C-KIT突变(66%),无一例在血小板衍生生长因子受体α(PDGFRA)中有突变,33%为非KIT/PDGFRA。小GIST的发生率随年龄增长而增加。在随访期间,没有小GIST出现复发,也没有GIST特异性死亡。挪威、冰岛和韩国的其他基于人群的研究证实,报告的小GIST发生率占所有GIST的四分之一。报告有丝分裂指数的研究的共同之处在于,几乎普遍发现小GIST的风险极低,复发极少,并且在几个系列中报告的长期生存率极佳,达到95%至100%。

讨论

每诊断出的四名GIST患者中就有一名是小GIST(<2厘米)。小GIST在老年人群中越来越常见,总体预后良好。为了避免过度治疗和治疗不足,需要确定真正的风险特征,以制定适当的监测和治疗策略。

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