Gastrointestinal carcinoids: Characterization by site of origin and hormone production

Objective To describe a large series of patients with carcinoid tumors in t erms of presenting symptoms, hormonal data, stage at diagnosis, pathologic features, and survival. Summary Background Data Published series have described significant prognos tic features of carcinoid tumors as site of origin, age, sex, stage at diag nosis, presence of high hormone levels, and increased T stage. Of these, st age at diagnosis and T stage seem to emerge most often as independent predi ctors of survival in multivariate analyses. Of carcinoid tumors, those aris ing from a midgut location have higher levels of serotonin and serotonin br eakdown products, as well as more frequent metastatic disease at presentati on, than those arising from either foregut or hindgut locations. Methods A prospective database-of carcinoid patients seen at Duke Universit y Medical Center was kept from 1970 to the present. Retrospective medical r ecord review was performed on this database to record presenting symptoms, hormonal data, pathologic features, and survival. Statistical methods inclu ded analysis of variance, Kaplan-Meier analysis, and Mantel-Cox proportiona l hazard survival analysis, with P < .05 considered significant for all tes ts. Results Carcinoids arising in different locations had different presentatio ns: rectal carcinoids presented significantly more often with gastrointesti nal bleeding, and midgut carcinoids presented significantly more often with flushing, diarrhea, and the carcinoid syndrome. Patients with midgut tumor s had significantly higher levels of serotonin and serotonin breakdown prod ucts, corresponding to higher metastatic tumor burdens. Although age, stage , region of origin, and urinary level of 5-hydroxyin-doleacetic acid predic ted survival by univariate analysis, only the latter three were independent predictors of survival by multivariate analysis. Of the patients with meta static disease at diagnosis, those with midgut tumors had better survival t han those with foregut or hindgut tumors. Conclusions Although region of origin is certainly an important factor in d etermination of prognosis, stage of disease at presentation is more predict ive of survival. Pancreatic and midgut carcinoids are metastatic at diagnos is more often than those arising in other locations, leading to a worse ove rall prognosis. Among patients with distant metastases, patients with midgu t primary tumors have improved survival despite increased hormone productio n compared with patients with tumors arising in other primary sites.