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文献考察：虫垂カルチノイド腫瘍
１）【虫垂疾患とその周辺】 虫垂疾患の各論 虫垂カルチノイド
Author：冲永功太(帝京大学 第2外科)
Source：臨床消化器内科(0911-601X)14巻11号 Page1505-1510(1999.09)
要旨：虫垂のカルチノイドは，虫垂の神経内分泌細胞から発生し，一般人口100，000人中約1．5人とまれな腫瘍である．消化管のカルチノイドのなかで，わが国では約12％を占めるが，欧米ではもっとも頻度が高い．カルチノイド症状を示す例はまれであり，急性虫垂炎として手術される場合やほかの開腹術で同時に切除され，病理組織診断にてカルチノイドと診断される症例が多い．80％の症例では腫瘍径1cm以下であり，75％の症例で腫瘍は先端に位置する．通常は悪性度は低く，虫垂切除術で十分である．しかし，腫瘍の径が2cmを超える症例や，壁外に浸潤やリンパ節転移を疑われる症例には，右半結腸切除の適応となる．虫垂に限局した症例の予後は良好であり，5年生存率は約94％である．
追記：カルチノイド腫瘍はserotonin，種々の消化管ホルモンやその前駆物質を産生する．カルチノイド症候群はそれら物質による顔面紅潮，発汗，下痢や腹痛などが典型的症状とされるが，カルチノイド腫瘍の10％程度にみられる．
２）Am J Gastroenterol. 1998 Mar;93(3):422-8.
A retrospective analysis of 1570 appendiceal carcinoids.
Sandor A, Modlin IM.
OBJECTIVE: Information about the management and outcome of appendiceal carcinoids is sparse, because few series comprise more than 100 cases. In this study we have analyzed the epidemiology of 1570 appendiceal carcinoids, to compare outcome with other gastrointestinal carcinoid tumors. METHODS: We evaluated 1570 appendiceal carcinoids in a series of 8305 carcinoid tumors from the SEER, the End Results Group, and the Third National Cancer Survey programs of the National Cancer Institute over the time period 1950-1991. RESULTS: Appendiceal carcinoids comprised 18.9% of all carcinoid tumors and exhibited a marked female predominance (M/F ratio: 0.47). Age-adjusted incidence rates were 1.7-fold higher in women compared to men. Appendiceal carcinoids present earlier (average age: 42.2 yr) than other gastrointestinal carcinoids (62.9 yr) or noncarcinoid appendiceal tumors (61.9 yr). At the time of diagnosis 35.4% were nonlocalized. The overall 5-yr survival for localized lesions was 94%, for regional invasion 84.6%, and for distant metastases 33.7%. The 5-yr survival of appendiceal carcinoids (85.9%) was the highest among all types of carcinoid tumors. In 14.6% noncarcinoid tumors at other sites were also evident. CONCLUSION: The high relative incidence of carcinoid tumors in the appendix is still poorly understood. The good overall 5-yr survival rates of appendiceal carcinoids as opposed to other carcinoids represents either a different biological behavior, earlier diagnosis, or expeditious management (appendectomy). However, the increased likelihood of coexisting neoplasms and the not uncommon presentation of metastatic disease should warrant careful evaluation and postoperative follow-up of such lesions.PMID: 9517651
３）Cancer. 1997 Feb 15;79(4):813-29.
An analysis of 8305 cases of carcinoid tumors.
Modlin IM, Sandor A.
BACKGROUND: Carcinoid tumors are unusual and most reports are anecdotal or limited in number. A series of 2837 cases was published in 1975. No recent large series is available. METHODS: The authors evaluated 5468 cases identified by the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute (NCI) from 1973 to 1991 together with 2837 carcinoid cases previously registered by 2 earlier NCI programs. To the authors' knowledge, the 8305 carcinoid tumors analyzed represent the largest current epidemiology series to date. RESULTS: The most frequent sites for carcinoids were the gastrointestinal (GI) tract (73.7%) and the bronchopulmonary system (25.1%). Within the GI tract, most occurred in the small bowel (28.7%), appendix (18.9%), and rectum (12.6%). For all sites, age-adjusted incidence rates were highest in African American males (2.12 per 100,000 population per year). Associated noncarcinoid tumors were frequent in conjunction with small intestinal (16.6%), appendiceal (14.6%), and colonic carcinoids (13.1%). The highest percentage of nonlocalized lesions were noted for pancreatic (76.1%), colonic (71.2%), and small intestinal carcinoids (70.7%) and this corresponded to their poor 5-year survival rates (34.1%, 41.6%, and 55.4%, respectively). The best 5-year survival rates were recorded for appendiceal (85.9%), bronchopulmonary (76.6%), and rectal carcinoids (72.2%). These exhibited invasive growth or metastatic spread in only 35.4%, 27.2%, and 14.2% of cases, respectively. CONCLUSIONS: Carcinoids appear to have increased in incidence in the past 20 years. In part, this may be due to different surgical rules of the various registries, improved diagnostic technology, and increased awareness. A cumulative analysis of all types of carcinoid tumors in the SEER group indicates that in 45.3% metastases are already evident at the time of diagnosis. The overall 5-year survival rate of all carcinoid tumors regardless of site was 50.4% +/- 6.4%.PMID: 9024720
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