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

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Jul 6, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Goblet Cell Carcinoid/Mixed Adenocarcinoma-Carcinoid

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