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Appendix

Other tumors

Goblet cell carcinoid


Reviewer: Jaleh Mansouri, M.D. (see Reviewers page)
Revised: 21 October 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

See also Carcinoid and L cell carcinoid

General
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● Also called GCC, mucinous carcinoid, adenocarcinoid, microglandular goblet cell carcinoma, crypt cell carcinoma
● Shows divergent differentiation of normal crypt stem cells (columnar, endocrine, goblet cell, Paneth cell lineages) between typical carcinoma and adenocarcinoma
● Less common than classic carcinoids (6% of all appendiceal carcinoids), usually older patients

Clinical features
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● 5 year survival is 73-84%
● Up to 20% spread beyond appendix; often to ovaries or peritoneal surfaces
● Metastases may have goblet cell carcinoid, signet ring cell carcinoma or classical carcinoid histology
● Up to 13% of patients die of tumor (intermediate prognosis between classical carcinoid and adenocarcinoma)
● Poor prognostic factors: solid pattern or carcinomatous pattern in 50%+ of tumor; also perineural invasion, lymphatic invasion, spread beyond appendix, serosal involvement at presentation, incomplete excision at appendix base
● A few cases of combined classic carcinoid and goblet cell carcinoid (GCC) tumor have been described (morphologic variant believed to represent dual parallel differentiation although collision tumor must also be considered as a possibility, Am J Surg Pathol 2010;34:1163)
● May have associated frank carcinomatous component (mixed goblet cell carcinoid-adenocarcinomas); some authors recommend processing of entire appendix to determine extent of carcinomatous component

Treatment
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● Appendectomy, right hemicolectomy if predominant carcinomatous growth pattern, significant mitotic activity or tumor beyond appendiceal wall (Am J Surg Pathol 2008;32:1429)

Gross description
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● Thickened appendiceal wall
● Usually no gross tumor

Micro description
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● Usually spares mucosa and infiltrates muscularis propria and periappendiceal fat
● Solid tumor cell clusters, crypt-like structures or tubules of mucus-secreting cells distended with mucin resembling goblet cells or signet ring cells, but also with eosinophilic cytoplasm resembling classic carcinoid in some cells (World J Gastrointest Oncol 2010;2:251)
● Nuclei are bland and monomorphic
● May have pools of extracellular mucin
● Scattered Paneth cells in tumors with crypt like structures
● Extensive perineural invasion common
● 25% of tumor is single cells

Carcinomatous growth pattern:
● Cribriform growth pattern, solid sheets of infiltrating single (signet) ring cells
● Nuclear pleomorphism with frequent mitotic activity (2+ mitotic figures/10 HPF)

Micro images
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Various images

Molecular description
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● Loss of heterozygosity of 18q (56%), 16q (38%), 11q (25%), resemble ileal carcinoids more than appendiceal adenocarcinoma (Mod Pathol 2003;16:1189)
● No Kras mutations

Electron microscopy description
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● Mucinous vacuoles, membrane bound neurosecretory granules

Electron microscopy images
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Electron dense granules (arrows) and mucin granules

Positive stains
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● Mucin (mucicarmine, PAS, PAS diastase, Alcian-blue), CEA, cytokeratin (especially CK20), lysozyme, chromogranin A, serotonin, synaptophysin (usually)

Differential diagnosis
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● Metastatic signet-ring carcinoma: infiltrative, signet-ring cells
Classic carcinoid tumor: no goblet cell differentiation, no intracellular mucus vacuoles although may have luminal mucus-lining neoplastic tubules, no Paneth cell differentiation
Mucinous adenocarcinoma: more aggressive, irregular fused structures without distinct lumina, often merges with mucosal adenoma (Arch Pathol Lab Med 1979;103:180)

End of Appendix > Other tumors > Goblet cell carcinoid (GCC)


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