Gastrointestinal stromal tumors:: The incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era -: A population-based study in western Sweden
Gastrointestinal stromal tumors:: The incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era -: A population-based study in western Sweden
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DOI：
10.1002/cncr.20862
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发表时间：
2005-02-15
期刊：
影响因子：
6.2
通讯作者：
Kindblom, LG
中科院分区：
文献类型：
--
作者：
Nilsson, B;Bümming, P;Kindblom, LG
背景资料。最近关于胃肠道间质瘤(GIST)及其发病机制的突破重新定义了诊断标准，并导致了分子靶向药物治疗的发展。新的治疗方案要求更准确的有关GIST的发病率、患病率、临床行为和预后因素的信息。方法：回顾了1983年至2000年瑞典西部(人口130-160万)所有可能被诊断为GIST的患者(n=1460)，其中288名患者被确定为原发GIST。结果：90%的胃肠道间质瘤是临床发现的(69%)或手术中偶然发现的(21%)；其余10%的胃肠道间质瘤是在尸检中发现的。在临床发现的有症状的GIST中，44%被归类为高风险(29%)或明显恶性(15%)，与肿瘤相关的死亡分别发生在63%和83%的患者中(估计的中位生存期分别为40个月和16个月)。与肿瘤相关的死亡仅发生在极低风险、低风险或中等风险肿瘤的170名患者中的2名(1.2%)。胃肠道间质瘤的年发病率为14.5‰。所有GIST风险组的患病率为129‰(高危组和恶性组为31‰)。结论GIST一直被低估：其发病率、患病率和临床侵袭性也被低估。目前，现有的基于肿瘤大小和有丝分裂率的风险组分层系统将预后较差的GIST患者区分开来。GIST患者的预后可以使用仅基于肿瘤大小和增殖指数的风险评分来改进。(C)2005年美国癌症协会。
BACKGROUND. Recent breakthroughs regarding gastrointestinal stromal tumors (GIST) and their pathogenesis have redefined diagnostic criteria and have led to the development of molecularly targeted drug therapy. New treatment options mandate more accurate information regarding the incidence, prevalence, clinical behavior, and prognostic factors of GIST.METHODS. All patients (n = 1460) who potentially had GIST diagnosed from 1983 to 2000 in western Sweden (population, 1.3-1.6 million) were reviewed, and 288 patients with primary GIST were identified. The incidence and prevalence of GIST were determined, and predictive prognostic factors, including current risk-group stratifications, were analyzed statistically.RESULTS. Ninety percent of GISTs were detected clinically due to symptoms (69%) or were incidental findings at surgery (21%); the remaining 10% of GISTs were found at autopsy. Forty-four percent of symptomatic, clinically detected GISTs were categorized as high risk (29%) or overtly malignant (15%), with tumor-related deaths occurring in 63% of patients and 83% of patients, respectively (estimated median survival, of 40 months and 16 months, respectively). Tumor-related deaths occurred in only 2 of 170 of patients (1.2%) with very-low-risk, low-risk, or intermediate-risk tumors. The annual incidence of GIST was 14.5 per million. The prevalence of all GIST risk groups was 129 per million (31 per million for the high-risk group and the overtly malignant group).CONCLUSIONS. GIST has been under recognized: Its incidence, prevalence, and clinical aggressiveness also have been underestimated. Currently existing risk-group stratification systems based on tumor size and mitotic rate delineate GIST patients who have a poor prognosis. Prognostication in patients with GIST can be refined using a proposed risk score based solely on tumor size and proliferative index. (C) 2005 American Cancer Society.