A 5‐decade analysis of 13,715 carcinoid tumors

• Published in: Cancer Vol. 97; no. 4; pp. 934 - 959
• Main Authors: Modlin, Irvin M., Lye, Kevin D., Kidd, Mark
• Format: Journal Article
• Language: English
• Published: New York Wiley Subscription Services, Inc., A Wiley Company 15.02.2003; Wiley-Liss
• Subjects: appendix; Biological and medical sciences; carcinoid; Carcinoid Tumor - epidemiology; colon; Continental Population Groups; Databases as Topic; epidemiology; Female; Gastroenterology. Liver. Pancreas. Abdomen; Gastrointestinal Neoplasms - epidemiology; Humans; Incidence; Male; Medical sciences; metastases; Middle Aged; Neoplasm Metastasis; Pneumology; Sex Factors; small bowel; stomach; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; survival; Survival Rate; Time Factors; Tumors; Tumors of the respiratory system and mediastinum; United States; North America; America; Human; Lung disease; Prognosis; appendix; Respiratory disease; survival; stomach; Malignant tumor; Gastrointestinal; Carcinoid tumor; carcinoid; Epidemiology; Bronchopulmonary; Five year survival; Incidence; metastases; Register; colon; Digestive diseases; Bronchus disease; Intestinal disease; small bowel; Public health; Gastric disease
• DOI: 10.1002/cncr.11105
• ISSN: 0008-543X

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. Cancer 2003;97:934–59. © 2003 American Cancer Society. DOI 10.1002/cncr.11105 Analysis of the largest known accumulation of carcinoid tumors reveals changing trends in the incidence of these tumors in sites such as the stomach and the appendix. Examination of incidence by race and gender suggests a genetic predisposition for carcinoid tumors at certain sites within certain groups.