Background:Gastrointestinal carcinoid (CAR) tumors are rare neuroendocrine tumors with unpredictable or indolent biology. Many can present with advanced disease, but more and more diagnoses are being made without metastatic disease. Currently, no staging system exists for these tumors thus limiting the ability to combare various treatment modalities across centers. Thus the goal of this study was to establish a staging system for CAR tumors consistent with the AJCC Staging Systems using the TNM strategy.
Methods:A retrospective review of our prospective database of 990 hepato-pancreatico-biliary patients and our tumor registry identified 108 patients identified with any gastrointestinal CAR tumors from 6/90-9/06. Pathological and clinical data was available to classify patients into our newly developed staging system. A staging system similar to other gastrointestinal cancers was used as the foundation with Tumor stage defined as depth of penetration or size of tumor, Nodal as presence/absence of nodes, and M metastatic. Kaplan Meier survival curves where then performed on this data according to delineate sensitivity.
Results: Patients were found to be Stage 0(Tis)11%, Stage 1 21%,Stage 2 8%,Stage 3 12%,and Stage 4 46%. There were 42 men and 66 women, 82 were Caucasian and 19 were African American, with a median age of 58 years (range 19-88 years). Primary tumors included 53 small bowel, 15 colon, 19 rectum, 9 stomach, and 7 unknown primary origin. The use of our initial staging system demonstrated a trend in differences in survival across all 4 stages (Figure 1).
Conclusion: The use of our initial staging proposal accurately delineates the biology of the disease with accurate overall survival estimates. The addition of a dedicated AJCC staging system is needed for gastrointestinal carcinoids. Widespread use of this staging system may contribute to the future management and treatment of gastrointestinal carcinoid tumors.

Overall Survival by stage of gastrointestinal carcinoid