Grade 3 tumors: Poorly differentiated neuroendocrine carcinoma (small or large cell type)
TERMINOLOGY
Abbreviations
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Well-differentiated neuroendocrine neoplasm (WDNEN)
Synonyms
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Well-differentiated neuroendocrine tumor
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Carcinoid tumor (not preferred)
Definitions
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Low- to intermediate-grade neoplasms with predominantly neuroendocrine differentiation arising in ampulla of Vater and periampullary region
ETIOLOGY/PATHOGENESIS
Disease Association
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Neurofibromatosis type 1
Somatostatin-producing tumors are most common
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Multiple endocrine neoplasia type 1
Gastrin-producing tumors are most common
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Helicobacter pylori infection
CLINICAL ISSUES
Epidemiology
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Incidence
< 1% of all gastrointestinal neuroendocrine neoplasms
< 2% of all tumors of ampullary region