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Ampulla of Vater


Neuroendocrine carcinoma

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 21 August 2014, last major update August 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.


● Rare, present with progressing jaundice
● Aggressive with poor prognosis (Hepatobiliary Pancreat Dis Int 2008;7:422)

Case reports

● 50 year old man with obstructive jaundice with an ampullary mass: collision tumor-mixed adenoneuroendocrine carcinoma (Case of the Week #322)
● 55 year old woman with large cell neuroendocrine carcinoma with glandular differentiation (J Clin Pathol 2004;57:1098)
● 73 year old woman with large cell neuroendocrine carcinoma with squamous cell and glandular components (Jpn J Clin Oncol 2011;41:434)
● 74 year old man with small cell neuroendocrine carcinoma with villous adenoma (World J Gastroenterol 2008;14:4709)
● 74 year old with large cell neuroendocrine carcinoma (Arch Pathol Lab Med 2003;127:221)

Micro description

Large cell tumor:
● Islands and trabeculae of large cells with brisk mitotic activity and extensive necrosis
● Cells have more cytoplasm than small cell carcinoma, irregular chromatin, frequent nucleoli

Micro images

Large cell neuroendocrine carcinoma

Small cell neuroendocrine carcinoma

Case of the Week #322:

Line 2: CDX2 (left); chromogranin (middle); synaptophysin (right)

Positive stains

Large cell tumor: cytokeratin, chromogranin, synaptophysin, neuron-specific enolase

Differential diagnosis

● Carcinoid
● Small cell carcinoma
● Lymphoma
● Poorly differentiated carcinoma
● Metastases

End of Ampulla of Vater > Carcinoma > Neuroendocrine carcinoma

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