Well-differentiated Neuroendocrine Neoplasm, Ampulla



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






A well-circumscribed, well-differentiated neuroendocrine tumor is seen involving the distal portion of the bile duct. The lumen of the bile duct image is narrowed.






A gastrinoma of the periampullary region infiltrates the overlying duodenal mucosa image. The neoplastic cells in this neuroendocrine tumor are monotonous.


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

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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Well-differentiated Neuroendocrine Neoplasm, Ampulla

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