Clinicopathologic feature and outcome of appendiceal goblet cell carcinoid and neuroendocrine tumor.

Abstract

e14170 Background: Goblet cell carcinoid (GCC) of the appendix is a rare tumor comprising 5% of primary appendiceal tumors. GCC shares histological features of both neuroendocrine tumor (NET) and adenocarcinoma. Clinically, GCC also has an intermediate behavior between them. The management of GCC is mostly based on small retrospective series. We report the clinicopathological features, treatments, and outcomes of patients with appendiceal GCCs and NETs from a merged database between Emory University and the Ohio State University. Methods: We identified patients with appendiceal GCCs and NETs treated at these institutions over the period of 12 years from 1999 to 2011. Chart reviews for age, gender, date of diagnosis, TNM stage, chemotherapy, intraperitoneal hyperthermic perfusion (IPHP), surgery, and survival were performed. Results: See table. In the GCC patients with stage I, II, and III, 30 received surgery alone and 1 received surgery plus IPHP. 29 out of 31 (94%) patients are alive without disease recurrence. Out of 6 GCC patients with stage IV, 5 received chemotherapy, 1 received chemotherapy plus IPHP, and 2 (33%) are alive. In the NET patients with stage I,II, and III, all 29 were treated with surgery alone. 29 out of 31 patients are alive and there was no disease-related mortality. Out of 2 NET patients with stage IV, one received chemotherapy and both are alive. Conclusions: Our data confirms the excellent prognosis of appendiceal NET regardless of stage. The outcomes of surgery for patients with stage I, II, and III in both GCCs and NETs were excellent. Patients with stage IV GCCs have higher possibility of aggressive clinical courses. Chemotherapy or IPHP should be considered in the management of stage IV GCCs. [Table: see text]