AbstractBackground and ObjectivesGoblet cell carcinoid, a rare tumor of intermediate malignant potential, is known to account for a significant minority of appendiceal neoplasms. Sixteen new cases of gastrointestinal goblet cell carcinoid were reviewed to describe their presentation, treatment, and outcome.MethodsA review of 16 cases from a single institution.ResultsSixteen patients were diagnosed with goblet cell carcinoid between 1995 and 2005. Presenting diagnoses included appendicitis (n = 8), abdominal or liver mass (n = 5), uterine fibroids (n = 1), ovarian mass (n = 1), and Crohn's Disease exacerbation (n = 1). Mean follow‐up was 12 months with a mortality of 19% (n = 3). Patients were divided into two groups: those where the diagnosis was an incidental finding at operation (Group 1) and those where the presentation was of an abdominal mass or metastatic disease (Group 2). Nine of ten patients in Group 1 initially received appendectomies. Group 2 included patients presenting with Krukenberg type lesions (n = 2) and abdominal masses (n = 4).ConclusionsGoblet cell carcinoid is a rare malignant tumor largely affecting the appendix. In patients presenting with appendicitis, our series does not support the recommendation of right hemicolectomy based on pathologic diagnosis alone and surgical intervention must be customized to the individual patient. J. Surg. Oncol. 2006;94:396–402. © 2006 Wiley‐Liss, Inc.