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Endocrine Abstracts (2018) 56 EP3 | DOI: 10.1530/endoabs.56.EP3

1Aizu Chuo Hospital, Aizu Wakamatsu, Japan; 2Tohoku University of Medicine, Sendai, Japan.


Background: Neuroendocrine tumors (NETs) of the ampulla of Vater are rare and sometimes difficult to diagnose preoperatively. We report a case of neuroendocrine carcinoma (NEC) of the ampulla of Vater diagnosed by endoscopic biopsy.

Case presentation: The patient was a 83-year-old male complaining of jaundice. Endoscopic retrograde cholangiopancreatography (ERCP) was performed. It revealed a protruding tumor in the major papilla with a diameter of 18mm, and a obstruction of lower bile duct. Endoscopic biopsies from the ulcerative region, and endoscopic retrograde biliary drainege (ERBD) was performed. Pathological findings of the endoscopic biopsies revealed a poorly-differentiated tumor. Immunohistochemically, the biopsied specimens stained positive for chromogranin A, CD56, synaptophysin, and AE1/AE3. The Ki-67-labeling index was 80%. The final preoperative diagnosis was NEC G3. Enhanced abdominal computed tomography (CT) scan revealed a 17 mm slightly enhanced tumor at the ampulla of Vater and some regional lymph node swelling. Pre-operative diagnosis based on CT was difficult since it presented with a similar clinical picture to the adenocarcinomas of this region. Pancreaticoduodenectomy with regional lymph node dissection was performed. Pathological examination revealed a poorly differentiated carcinoma in the region of the major papilla, with a diameter of 20×18 mm. Lymph node on the posterior surface of the pancreatic head (#13) was positive. TNM staging was T2 N1 M0, StageIIb. The immunohistochemical study showed positive for chromogranin A, CD56, Neuron specific enolase (NSE), and synaptophysin. The mitotic index was seven mitoses in ten HPF and Ki-67 was 50%. First-line chemothepapy for the patient might be platinum-based regimens. Because the patient was 83-year-old, postoperative chemotherapy has not performed. Post operative course was uneventful and the patient discharged 24 days after the operation.

Volume 56

20th European Congress of Endocrinology

Barcelona, Spain
19 May 2018 - 22 May 2018

European Society of Endocrinology 

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