Appendiceal goblet cell carcinoid: common errors in staging and clinical interpretation with a proposal for an improved terminology

Hum Pathol. 2017 Jul;65:187-193. doi: 10.1016/j.humpath.2017.05.012. Epub 2017 May 24.


Goblet cell carcinoid (GCC) is staged and treated as adenocarcinoma (AC) and not as neuroendocrine tumor (NET) or neuroendocrine carcinoma. The term carcinoid may lead to incorrect interpretation as NET. The aim of the study was to explore pitfalls in staging and clinical interpretation of GCC and mixed GCC-AC, and propose strategies to avoid common errors. Diagnostic terminology, staging, and clinical interpretation were evaluated in 58 cases (27 GCCs, 31 mixed GCC-ACs). Opinions were collected from 23 pathologists using a survey. Clinical notes were reviewed to assess the interpretation of pathology diagnoses by oncologists. NET staging was incorrectly used for 25% of GCCs and 5% of mixed GCC-ACs. In the survey, 43% of pathologists incorrectly indicated that NET staging is applicable to GCCs, and 43% incorrectly responded that Ki-67 proliferation index is necessary for GCC grading. Two cases each of GCC and mixed GCC-AC were incorrectly interpreted as neuroendocrine neoplasms by oncologists, and platinum-based therapy was considered for 2 GCC-AC cases because of the mistaken impression of neuroendocrine carcinoma created by use of the World Health Organization 2010 term mixed adenoneuroendocrine carcinoma. The term carcinoid in GCC and use of mixed adenoneuroendocrine carcinoma for mixed GCC-AC lead to errors in staging and treatment. We propose that goblet cell carcinoid should be changed to goblet cell carcinoma, whereas GCC with AC should be referred to as mixed GCC-AC with a comment about the proportion of each component and the histologic subtype of AC. This terminology will facilitate appropriate staging and clinical management, and avoid errors in interpretation.

Keywords: Adenocarcinoma; Appendix; Goblet cell carcinoid; MANEC; Mixed adenoneuroendocrine carcinoma; Neuroendocrine tumor; Terminology.

MeSH terms

  • Adenocarcinoma / chemistry
  • Adenocarcinoma / classification
  • Adenocarcinoma / pathology*
  • Appendiceal Neoplasms / chemistry
  • Appendiceal Neoplasms / classification
  • Appendiceal Neoplasms / pathology*
  • Carcinoid Tumor / chemistry
  • Carcinoid Tumor / classification
  • Carcinoid Tumor / pathology*
  • Carcinoma, Neuroendocrine / chemistry
  • Carcinoma, Neuroendocrine / classification
  • Carcinoma, Neuroendocrine / pathology*
  • Consensus
  • Databases, Factual
  • Diagnosis, Differential
  • Diagnostic Errors
  • Humans
  • Immunohistochemistry
  • Ki-67 Antigen / analysis
  • Neoplasm Staging
  • Neoplasms, Complex and Mixed / chemistry
  • Neoplasms, Complex and Mixed / classification
  • Neoplasms, Complex and Mixed / pathology*
  • Predictive Value of Tests
  • Retrospective Studies
  • Surveys and Questionnaires
  • Terminology as Topic*


  • Ki-67 Antigen