Objective To explore the clinicopathologic features,diagnostic criteria and clinical prognostic factors of Goblet cell carcinoid (GCC) of the appendix.Methods The clinical and pathological data from 6 GCC patients were analyzed including age,clinical stage,surgical procedure,outcome,macroscopic features,histological sections and immunohistochemistry.Results The median age was 49.67 years old.Clinically,4 patients presented the signs and symptoms of acute appendicitis,while 2 patients presented the signs and symptoms of abdominal pain.4 patients of them undergone the simple appendectomy,the other 2 undergone the right hemicolectomy.All patients who had undergone the operation treatment survived.One patient was lost to the follow-up.Macroscopically,no masses were found in the appendix.Microscopically,4 cases showed that the tumor cells were identical to the goblet cell normal small intestinal crypts morphologically.The atypia,necrosis and mitotic figures of neoplasm cells were absent.2 cases were composed of small,discrete acini,tubules lined by a single layer of cuboidal or columnar cell,the signet-ring cells were uniform with pattern of infiltration being nests,rosrttes,or clumps without a dintinct lumen.All GCC cases were positive to Syn,4 cases were positive to CgA,some cases expressed NSE,CD56,CK7,CK20,Ki-67 index of 5 cases was under 2 ％,the other 1 was 3 ％.None had lymph node metastases,intestinal metastases or ovary metastases.Conclusions GCC of appendix is a rare neoplasm,and has more aggressive behaviour than classic carcinoids.Positive expression of Syn and CgA is necessory for the diagnosis of GCC,Ki-67 index may suggest the grading of tumor.