Medline ® Abstracts for References 4,61
of 'Cancer of the appendix and pseudomyxoma peritonei'
Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973-1998.
McCusker ME, CotéTR, Clegg LX, Sobin LH
BACKGROUND: Cancer of the appendix is an uncommon disease that is rarely suspected rarely before surgery. Although several case series of these tumors have been published, little research has been anchored in population-based data on cancer of the appendix.
METHODS: This analysis included all actively followed cases of appendiceal neoplasms reported to the National Cancer Institute's Surveillance, Epidemiology and End-Results (SEER) program between 1973 and 1998. Tumors were classified as "colonic type" adenocarcinoma, mucinous adenocarcinoma, signet ring cell carcinoma, goblet cell carcinoid, and "malignant carcinoid" (SEER only collects data on carcinoids specifically classified as malignant). We compared incidence, overall survival and survival rates by extent of disease at diagnosis.
RESULTS: Between 1973 and 1998, 2117 appendiceal malignancies were reported to the SEER program, of which 1645 cases were included in the analysis. Age-adjusted incidence of cancer of the appendix was 0.12 cases per 1,000,000 people per year. Demographic characteristicsof patients with goblet cell carcinoid tumors were midway between those of patients with malignant carcinoid and all types of adenocarcinomas. After controlling for age and extent of disease at diagnosis, the overall survival rate for patients diagnosed between 1983 and 1997 (n = 1061) was significantly worse for those with signet ring cell carcinoma than for those with any other tumor type (P<0.01). In addition, overall survival rates were better for patients with malignant carcinoid (P = 0.01).
CONCLUSIONS: Demographic characteristics of patients with cancer of the appendix vary by histology. Except for signet ring cell carcinoma and malignant carcinoid, the extent of disease at time of diagnosis is a more important predictor of survival than histology.
Department of Epidemiology and Preventive Medicine, University of Maryland, Baltimore, Maryland 21201, USA. firstname.lastname@example.org
Goblet cell carcinoid of the appendix.
Butler JA, Houshiar A, Lin F, Wilson SE
Am J Surg. 1994;168(6):685.
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.
Department of Surgery, University of California, Irvine.