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.