A 5-decade analysis of 13,715 carcinoid tumors
A 5-decade analysis of 13,715 carcinoid tumors
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作者：
Modlin, IM;Lye, KD;Kidd, M
背景。类癌肿瘤代表具有蛋白质临床表现的异常且复杂的疾病谱。这项由1950年至1999年的患者组成的几个大型美国数据库的汇编检查了13,715个类癌肿瘤，并提供了有关自然历史的流行病学信息以及该实体检测和诊断。作者评估了1973年至1999年的国家癌症研究所（NCI）的监视，流行病学和最终结果（SEER）计划（SEER）鉴定的10,878个类癌肿瘤，此外还没有2837种类癌肿瘤，这些肿瘤先前已由两个早期的NCI计划进行了注册。据作者所知，这代表了目前最大的流行病学系列，该系列针对迄今为止的类癌肿瘤。确定了某些部位的类癌肿瘤发病率的特定趋势。在最近收集的数据子集中，表明类癌发生率最高的地点是胃肠道（67.5％）和支气管肺系统（25.3％）。在胃肠道内，大多数类癌肿瘤发生在小肠（41.8％），直肠（27.4％）和胃（8.7％）中。对于所有地点，黑人男性的年龄调整率最高（每100,000人每年4.48）。相关的非癌肿瘤与小肠道（29.0％）），胃（20.5％），结肠（20.0％）和阑尾（18.2％）类癌相结合。盲肠（81.5-83.2％）和胰腺（71.9-81.3％）的类癌被发现，非局部病变的百分比最高。 （65.4％）类癌。直肠（88.3％），支气管肺（73.5％）和阑尾（71.0％）类癌的患者记录了最佳的5年生存率；这些肿瘤分别在3.9％，27.5％和38.8％的患者中表现出侵入性生长或转移性扩散。在过去30年中，类癌的总发病率似乎有所增加；对于某些地点，这种趋势已经近半个世纪了。最近的胃癌和直肠癌的显着增加以及阑尾类癌发病率的同时下降可能部分归因于本报告中检查的数据库中的注册规则，或者是鉴于诊断技术的改善；对类癌肿瘤的认识和对类癌肿瘤的意识也可能发挥重要作用。在所有类癌的患者中，有12.9％的人在诊断时已经明显了遥远的转移。所有类癌肿瘤的总体5年生存率，无论现场如何，均为67.2％。这些发现质疑类癌疾病广泛颁布的相对良性。在美国境内的黑人和亚洲人群中，某些类癌（例如直肠的类癌）似乎过多，这表明遗传学在这种有趣的疾病发展中的作用。
BACKGROUND. Carcinoid tumors represent an unusual and complex disease spectrum with protean clinical manifestations. This compilation of several large United States-based databases comprising patients from 1950 to 1999 examines 13,715 carcinoid tumors and provides epidemiologic information regarding the natural history and evolution of the detection and diagnosis of this entity.METHODS. The authors evaluated 10,878 carcinoid tumors that were identified by the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute (NCI) from 1973 to 1999 in addition to 2837 carcinoid tumors that were registered previously by two earlier NCI programs. To the authors' knowledge, this represents the largest current epidemiology series addressing carcinoid tumors to date.RESULTS. Specific trends in incidence for carcinoid tumors of certain sites were identified. Among the most recently collected subset of data, sites that demonstrated the greatest incidence of carcinoids were the gastrointestinal tract (67.5%) and the bronchopulmonary system (25.3%). Within the gastrointestinal tract, most carcinoid tumors occurred in the small intestine (41.8%), rectum (27.4%), and stomach (8.7%). For all sites, age-adjusted incidence rates were highest in black males (4.48 per 100,000 population per year). Associated noncarcinoid tumors were frequent in conjunction with small intestinal (29.0%)), gastric (20.5%), colonic (20.0%), and appendiceal (18.2%) carcinoids. The highest percentages of nonlocalized lesions were noted for cecal (81.5-83.2%) and pancreatic (71.9-81.3%) carcinoids, whereas the highest percentage of localized disease was found among rectal (81.7%), gastric (67.5%), and bronchopulmonary (65.4%) carcinoids. The best 5-year survival rates were recorded for patients with rectal (88.3%), bronchopulmonary (73.5%), and appendiceal (71.0%) carcinoids; these tumors exhibited invasive growth or metastatic spread in 3.9%, 27.5%, and 38.8% of patients, respectively.CONCLUSIONS. Carcinoids appear to have increased in overall incidence over the past 30 years; for some sites, this trend has been evident for nearly half a century. Recent marked increases in gastric and rectal carcinoids and a concomitant decrease in appendiceal carcinoid incidence may be due in part to varying rules of registration among the compiled databases examined in this report or to improvements in diagnostic technology; increased awareness of and about carcinoid tumors also may play a significant role. In 12.9% of all patients with carcinoid, distant metastases already were evident at the time of diagnosis; the overall 5-year survival rate for all carcinoid tumors, regardless of site, was 67.2%. These findings bring into question the widely promulgated relative benignity of carcinoid disease. Certain carcinoid tumors, such as those of the rectum, appear to be over-represented among the black and Asian populations within the United States, suggesting the role of genetics in the development of this intriguing disease.