Well-differentiated Neuroendocrine Neoplasm, Ampulla
Vikram Deshpande, MD
Key Facts
Etiology/Pathogenesis
Syndromic cases associated with neurofibromatosis, MEN1, Zollinger-Ellison syndrome
Clinical Issues
Behavior/prognosis difficult to predict
Size, mitotic rate are poor predictors of prognosis
Microscopic Pathology
Similar to well-differentiated neuroendocrine neoplasms of other sites
Somatostatinoma
Gland formation common, some with psammoma bodies in lumen
Ancillary Tests
Majority stain for one or more peptides
TERMINOLOGY
Abbreviations
Well-differentiated neuroendocrine neoplasm (WDNEN)
Synonyms
Carcinoid tumor
ETIOLOGY/PATHOGENESIS
Syndromic Cases
Neurofibromatosis
Majority are somatostatinomas
MEN1 and Zollinger-Ellison syndrome
Gastrinoma or nonfunctional tumor
Nonsyndromic Cases
Unknown
CLINICAL ISSUES
Epidemiology
Gender
Male predominance
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