GOBLET CELL CARCINOID OF THE APPENDIX

BACKGROUND: Over the past 15 years, a variant of appendiceal carcinoid has been recognized which is not widely known to the general surgeon. This tumor, variously labeled adenocarcinoid or goblet cell carcinoid, has a more aggressive natural history than classic appendiceal carcinoids and requires a different surgical approach. PATIENTS AND METHODS: A survey of the tumor registries of 10 hospitals identified 7 female and 2 male patients with goblet cell carcinoid of the appendix between 1983 and 1993. The clinical data of these patients were reviewed. RESULTS: Median;age was 58 years (range 31 to 73). Clinical presentation followed two distinct patterns: acute onset suggestive of appendicitis or chronic symptoms associated with a pelvic mass. In no case was the diagnosis suspected preoperatively or established intraoperatively. Four patients with discrete distal appendiceal tumors treated with appendectomy are alive without evidence of disease 2 to 11 years since their diagnoses. The 5 patients with diffuse appendiceal involvement all had intra-abdominal metastases. Four are dead of disease at intervals from 5 months to 2 years postdiagnosis. CONCLUSIONS: Goblet cell carcinoids are a distinct clinical and pathologic entity with variable malignant potential. Patients with diffuse appendiceal involvement require aggressive surgery as part of multimodal therapy.