Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients

PLoS One. 2015 Feb 11;10(2):e0117627. doi: 10.1371/journal.pone.0117627. eCollection 2015.

Abstract

Background: Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features.

Patients and methods: Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013.

Results: Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. At diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p<0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002).

Conclusion: The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Carcinoid Tumor / diagnostic imaging
  • Carcinoid Tumor / epidemiology*
  • Carcinoid Tumor / pathology
  • Carcinoid Tumor / therapy*
  • Cohort Studies
  • Databases, Factual
  • Denmark / epidemiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neoplasm Staging
  • Postoperative Period
  • Prognosis
  • Radionuclide Imaging
  • Receptors, Somatostatin / metabolism
  • Retrospective Studies
  • Survival Analysis

Substances

  • Receptors, Somatostatin

Grant support

This work was supported by The Danish Research Council for Strategic Research, Rigshospitalet’s Research Foundation, The Research Foundation of the Capital Region, Arvid Nilssons Foundation, Internalization Foundation, The Lundbeck Foundation, Svend Andersen Foundation, National Advanced Technology Foundation and The NOVO Nordisk Foundation. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.