Goblet Cell Carcinoid/Mixed Adenocarcinoma-Carcinoid

Goblet Cell Carcinoid/Mixed Adenocarcinoma-Carcinoid
Scott R. Owens, MD
Key Facts
Terminology
  • Goblet cell carcinoid
    • Proliferation of mucin-producing cells with features of both neuroendocrine and crypt/glandular differentiation
Clinical Issues
  • Some suggest regional resection (right hemicolectomy) even for localized tumors
  • GCC more aggressive than conventional carcinoid tumor
  • MA-GCC behaves more like conventional adenocarcinoma
Macroscopic Features
  • GCC (especially MA-GCC) may spread directly to other organs &/or transperitoneally
  • Entire appendix should be submitted when GCC is suspected or found
Microscopic Pathology
  • Presence of goblet-like cells, Paneth cells, and enterochromaffin cells suggest “recapitulation” of normal crypt structure
Diagnostic Checklist
  • Careful evaluation of appendiceal margin and extent of infiltration through appendiceal wall is important
  • GCC/MA-GCC must be kept in mind as possible primary source when evaluating disseminated mucinous/signet-ring carcinomas
Hematoxylin & eosin low-power view shows appendiceal goblet cell carcinoid. Note the diffuse wall thickening. Individually infiltrating tumor nests image are difficult to see at this magnification.
Hematoxylin & eosin seen at high-power shows individual nests of mucin-containing cells that resemble goblet cells image.
TERMINOLOGY
Abbreviations
  • Goblet cell carcinoid (GCC)
  • Mixed adenocarcinoma-goblet cell carcinoid (MA-GCC)
  • Enterochromaffin cell (ECC)
  • Neuroendocrine (NE)
Synonyms
  • Adenocarcinoid
  • Crypt cell carcinoma
  • Microglandular carcinoma
  • Mucinous carcinoid
Definitions
  • GCC
    • Proliferation of mucin-producing cells with features of both NE and crypt/glandular differentiation
  • MA-GCC
    • More aggressive tumor in which unequivocal adenocarcinoma is found in conjunction with (presumably arising from) a GCC
ETIOLOGY/PATHOGENESIS
Unknown
  • Some genetic alterations noted
    • Loss of heterozygosity in 11q, 16q, and 18q
    • Mutations in KRAS, β-catenin, SMAD4 genes absent or low-level in some studies
CLINICAL ISSUES
Epidemiology
  • Incidence
    • Rare
  • Age
    • Occurs in older individuals than conventional carcinoid does
Presentation
  • Acute appendicitis
    • Abdominal pain
    • Diarrhea
  • Perforation
Treatment
  • Surgical approaches
    • Controversial
      • Appendectomy may be sufficient for tumors confined to appendix
      • Some suggest regional resection (right hemicolectomy) even for localized tumors
      • Regional resection important for aggressive tumors that involve proximal appendiceal margin &/or those with transmural extension
    • Transperitoneal spread treated as disseminated adenocarcinoma
      • Adjuvant chemotherapy advocated by some
Prognosis
  • GCC more aggressive than conventional carcinoid tumor
    • Behaves more like low-grade adenocarcinoma
      • Source of “crypt cell carcinoma” as alternate name
    • Best outcomes in tumors that are confined to appendix
    • 5-year survival reported to be around 75-85%
  • MA-GCC behaves more like conventional adenocarcinoma
    • Lower survival rates
MACROSCOPIC FEATURES
General Features
  • Most common by far in appendix but can sometimes occur elsewhere in gastrointestinal tract
  • Most often do not create discrete tumor mass
  • Diffuse wall thickening common
  • GCC (especially MA-GCC) may spread directly to other organs &/or transperitoneally throughout abdomen
    • May produce disseminated carcinoma strongly resembling signet-ring cell carcinoma
      • Ovarian involvement common (Krukenberg tumor-like appearance)
      • Possible pseudomyxoma peritonei-like presentation
Sections to Be Submitted
  • Entire appendix should be submitted if GCC is suspected or found
  • Careful evaluation of proximal margin particularly important
MICROSCOPIC PATHOLOGY
Key Descriptors
  • Predominant pattern/injury type
    • Neoplastic
    • Infiltrative
      • Concentric pattern of infiltration through appendiceal wall
      • Unique low-power microscopic appearance
      • Extensiveness of infiltration best seen on keratin or mucin stains
    • Mucinous
      • Distended mucin-filled cells resembling goblet cells
      • Pools of mucin occasionally present
    • Small islands and nested
      • Small round “microglandular” collections of goblet-like cells
      • True lumen formation is rare
      • May also form linear collections infiltrating through appendiceal wall
      • MA-GCC has frankly carcinomatous tubules (less mucin, more pleomorphic and malignant-appearing epithelial cytology)
  • Predominant cell/compartment type
    • Epithelial, mucinous
    • Epithelial, neuroendocrine
      • Enterochromaffin cells admixed with goblet cell-like epithelial cells
  • Histologic features
Tags:
Jul 6, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Goblet Cell Carcinoid/Mixed Adenocarcinoma-Carcinoid

Full access? Get Clinical Tree
Get Clinical Tree app for offline access