Objective To analyze the clinicopathological characteristics of appendiceal goblet cell adeno-carcinoma.Methods This study included 11 patients diagnosed with appendiceal goblet cell adenocarcinoma at the Department of Pathology,Xiamen University Affiliated First Hospital,Xiang'an Branch,between January 2020 and March 2024.All patients underwent immunohistochemical examination,and four cases also received genetic testing.Clinical data were collected to analyze the clinicopathological characteristics of appendiceal goblet cell adenocarcinoma.Results Clinical examination of the 11 patients revealed appendicitis-like gross appearance in all cases,While one case showed significant obstruction of the appendiceal lumen,one case had notable expansion at the appendiceal tip,and one case had dark brown serosal surface with perforation and adhesions.Among all cas-es,two cases involved the ileocecal region,one case involved the colonic wall,three cases invaded the mesentery,one case involved the duodenum and colon with implantation metastasis in the hepatorenal space,one case had lymph node metastasis,and three cases had metastasis to other locations.In terms of invasion,four cases invaded the subserosal layer of the appendix,three cases invaded the serosal layer,one case penetrated the serosa,and three cases had metastasis to other sites.Eight cases showed nerve invasion.There were ten high-grade cases and one low-grade case.Staging included one case of T3N0M0I1A,one case of PT3N2aMlb,and one case of stage IVc.Immunohistochemical results were CK7+in 3 cases,CK20+in 7 cases,CDX2+in 8 cases,syn+in 8 cases,CD56+in 4 cases,and CgA+in 5 cases.The Ki-67 prolifera-tion index ranged from 20%to 85%,with an average of 38.75%.Genetic mutations were detected in two cases:one had a G13D mutation in exon 2 of the KRAS gene,and the other had a Q61X mutation in exon 3 of the KRAS and NRAS genes.Conclusion Appendiceal goblet cell adenocarcinoma is a malignant tumor with a broad spectrum of morphological and biological behaviors,exhibiting high malignancy and invasiveness,especially in high-grade cases.It is often associated with other lesions and has non-specific clinical symptoms,which under-scores the need for clinicians and pathologists to enhance their understanding of the clinicopathological characteris-tics of appendiceal goblet cell adenocarcinoma to prevent misdiagnosis and missed diagnosis.