Varying malignant potential of appendiceal neuroendocrine tumors: Importance of histologic subtype

Cary Hsu, Asif Rashid, Yan Xing, Yi Ju Chiang, Ryaz B. Chagpar, Keith F. Fournier, George J. Chang, Y. Nancy You, Barry W. Feig, Janice N. Cormier

Research output: Contribution to journalArticlepeer-review

46 Scopus citations


Background: Neuroendocrine tumors (NETs) of the appendix include malignant carcinoid tumor (MCT), goblet cell carcinoid (GCT), and composite goblet cell carcinoid-adenocarcinoma (CGCC-A). Methods: We compared characteristics and outcomes of these histologic subtypes. Patients with appendiceal NETs were identified from the National Cancer Database (1998-2007). Descriptive statistics were used to compare cohorts and associations between clinicopathologic factors and overall survival (OS) were examined using Cox proportional hazards models. Results: A total of 2,812 patients with appendiceal NETs were identified. The most common histologic subtype was GCT (59.6%), followed by MCT (32.1%), CGCC-A (6.9%), and others (1.4%). CGCC-A had a significantly higher incidence of lymph node metastases (odds ratio [OR], 3.2; 95% confidence interval [CI], 2.1-4.8) and distant metastases (OR, 6.0; 95% CI = 3.8-9.3) than GCT. The 5-year OS was 86.3% (95% CI, 81.4-89.9) for MCT, 77.6% (95% CI, 74.0-80.8) for GCT, and 56.3% (95% CI, 42.1-68.4) for CGCC-A (P < 0.0001). Conclusion Appendiceal NETs represent a spectrum of disease with varying malignant potential: MCT (low), GCT (intermediate), and CGCC-A (high). GCTs represent the most common subtype, whereas CGCC-As place the patient at highest risk for regional and distant metastases and have the worst prognosis.

Original languageEnglish (US)
Pages (from-to)136-143
Number of pages8
JournalJournal of Surgical Oncology
Issue number2
StatePublished - Feb 2013


  • malignant potential
  • neuroendocrine tumors
  • tumors of the appendix

ASJC Scopus subject areas

  • Surgery
  • Oncology


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