U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ampulla of Vater Neuroendocrine Carcinoma

MedGen UID:
474113
•Concept ID:
C3272480
•
Neoplastic Process
Synonyms: Ampulla of Vater High Grade Neuroendocrine Carcinoma; Ampulla of Vater NEC; Ampulla of Vater NEC G3; Ampulla of Vater Poorly Differentiated Endocrine Carcinoma; Ampulla of Vater Small and Large Cell Endocrine Carcinoma; Neuroendocrine Carcinoma of the Ampullary Region

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the ampulla of Vater and the periampullary region. The mitotic count is more than 20 per 10 HPF. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma. Patients usually present with advanced stage disease. [from NCI]

Professional guidelines

PubMed

Clinicopathological Findings and Treatment Outcomes of Patients with Primary Hepatobiliary Neuroendocrine Neoplasms: A Retrospective Single-institution Analysis.
Komiyama S, Okusaka T, Maruki Y, Ohba A, Nagashio Y, Kondo S, Hijioka S, Morizane C, Ueno H, Sukeda A, Mizui T, Takamoto T, Nara S, Ban D, Esaki M, Hiraoka N, Shimada K
Intern Med 2024 Apr 1;63(7):891-901. Epub 2023 Aug 23 doi: 10.2169/internalmedicine.2016-23. PMID: 37612088Free PMC Article
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.
Mahansaria SS, Agrawal N, Arora A, Bihari C, Appukuttan M, Chattopadhyay TK
Int J Surg Pathol 2017 Oct;25(7):585-591. Epub 2017 May 29 doi: 10.1177/1066896917712454. PMID: 28552015

Recent clinical studies

Etiology

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
LONG-TERM FOLLOW-UP OF DUODENAL PAPILLARY SOMATOSTATINOMA TREATED BY ENDOSCOPIC PAPILLECTOMY.
Ardengh JC, Ardengh AO, Almeida AF, Machado MA
Arq Gastroenterol 2021 Jul-Sep;58(3):411-412. doi: 10.1590/S0004-2803.202100000-71. PMID: 34705982
Exosomal miRNA signatures of pancreatic lesions.
Vicentini C, Calore F, Nigita G, Fadda P, Simbolo M, Sperandio N, Luchini C, Lawlor RT, Croce CM, Corbo V, Fassan M, Scarpa A
BMC Gastroenterol 2020 May 6;20(1):137. doi: 10.1186/s12876-020-01287-y. PMID: 32375666Free PMC Article
Exocrine pancreatic insufficiency caused by a somatostatinoma of the minor and major duodenal papilla in a patient with neurofibromatosis.
Borobia FG, Fabregat J, Jorba R, Poves I, Biondo S, Serrano T, Figueras J, Jaurrieta E
Eur J Surg 2001 Feb;167(2):154-6. doi: 10.1080/110241501750070664. PMID: 11266260

Diagnosis

Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Endoscopic Ampullectomy of a Somatostatinoma.
Trindade AJ, Benias PC, Goldenberg S
Clin Gastroenterol Hepatol 2021 Mar;19(3):e24-e25. Epub 2020 Jan 15 doi: 10.1016/j.cgh.2020.01.010. PMID: 31954200
Clinical challenges and images in GI. Ampullary somatostatinoma associated with neurofibromatosis type 1, presenting with iron-deficiency anemia.
South CD, Baird MA, Marsh WL Jr
Gastroenterology 2009 Jan;136(1):51, 368. Epub 2008 Dec 6 doi: 10.1053/j.gastro.2008.05.028. PMID: 19063889
State of the art lecture: endoscopic ultrasound (EUS) and FNA in pancreatico-biliary tumors.
Chang KJ
Endoscopy 2006 Jun;38 Suppl 1:S56-60. doi: 10.1055/s-2006-946654. PMID: 16802226
Tumors of the major duodenal papilla.
Kim MH, Lee SK, Seo DW, Won SY, Lee SS, Min YI
Gastrointest Endosc 2001 Nov;54(5):609-20. doi: 10.1067/mge.2001.119254. PMID: 11677478

Therapy

The clinical profiles, management, and prognostic factors of biliary mixed neuroendocrine nonneuroendocrine neoplasms: A systematic review of the literature.
Wen LJ, Chen JH, Xu HJ, Yu Q, Deng Y, Liu K
Medicine (Baltimore) 2020 Dec 11;99(50):e23271. doi: 10.1097/MD.0000000000023271. PMID: 33327249Free PMC Article
Long-term complete remission of a patient with high grade neuroendocrine carcinoma of ampulla of Vater.
Taghizadeh-Hesary F, Moradi A, Malekzadeh M
BMJ Case Rep 2018 Jun 27;2018 doi: 10.1136/bcr-2018-224845. PMID: 29950363Free PMC Article
Surgical technique and results of total mesopancreas excision (TMpE) in pancreatic tumors.
Adham M, Singhirunnusorn J
Eur J Surg Oncol 2012 Apr;38(4):340-5. Epub 2012 Jan 20 doi: 10.1016/j.ejso.2011.12.015. PMID: 22264964
Cisplatin and etoposide as first-line chemotherapy for poorly differentiated neuroendocrine carcinoma of the hepatobiliary tract and pancreas.
Iwasa S, Morizane C, Okusaka T, Ueno H, Ikeda M, Kondo S, Tanaka T, Nakachi K, Mitsunaga S, Kojima Y, Hagihara A, Hiraoka N
Jpn J Clin Oncol 2010 Apr;40(4):313-8. Epub 2010 Jan 4 doi: 10.1093/jjco/hyp173. PMID: 20047862
Interventional endoscopy, neoadjuvant therapy and the gastroenterologist.
Snady H
Hematol Oncol Clin North Am 2002 Feb;16(1):53-79. doi: 10.1016/s0889-8588(01)00010-7. PMID: 12063829

Prognosis

Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
LONG-TERM FOLLOW-UP OF DUODENAL PAPILLARY SOMATOSTATINOMA TREATED BY ENDOSCOPIC PAPILLECTOMY.
Ardengh JC, Ardengh AO, Almeida AF, Machado MA
Arq Gastroenterol 2021 Jul-Sep;58(3):411-412. doi: 10.1590/S0004-2803.202100000-71. PMID: 34705982
Establishment of Patient-Derived Organoids and Drug Screening for Biliary Tract Carcinoma.
Saito Y, Muramatsu T, Kanai Y, Ojima H, Sukeda A, Hiraoka N, Arai E, Sugiyama Y, Matsuzaki J, Uchida R, Yoshikawa N, Furukawa R, Saito H
Cell Rep 2019 Apr 23;27(4):1265-1276.e4. doi: 10.1016/j.celrep.2019.03.088. PMID: 31018139
Large cell neuroendocrine carcinoma of the ampulla of Vater.
Beggs RE, Kelly ME, Eltayeb O, Crotty P, McDermott R, Ridgway PF
JOP 2012 Sep 10;13(5):470-5. doi: 10.6092/1590-8577/821. PMID: 22964952
Exocrine pancreatic insufficiency caused by a somatostatinoma of the minor and major duodenal papilla in a patient with neurofibromatosis.
Borobia FG, Fabregat J, Jorba R, Poves I, Biondo S, Serrano T, Figueras J, Jaurrieta E
Eur J Surg 2001 Feb;167(2):154-6. doi: 10.1080/110241501750070664. PMID: 11266260

Clinical prediction guides

Clinicopathological Findings and Treatment Outcomes of Patients with Primary Hepatobiliary Neuroendocrine Neoplasms: A Retrospective Single-institution Analysis.
Komiyama S, Okusaka T, Maruki Y, Ohba A, Nagashio Y, Kondo S, Hijioka S, Morizane C, Ueno H, Sukeda A, Mizui T, Takamoto T, Nara S, Ban D, Esaki M, Hiraoka N, Shimada K
Intern Med 2024 Apr 1;63(7):891-901. Epub 2023 Aug 23 doi: 10.2169/internalmedicine.2016-23. PMID: 37612088Free PMC Article
Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Mixed adenoneuroendocrine carcinomas of stomach and ampulla of vater after curative-intent resection: a single center cases series.
Yang S, Lu J, Cai Y, Li B, Xiong X
BMC Gastroenterol 2021 Aug 25;21(1):329. doi: 10.1186/s12876-021-01909-z. PMID: 34433421Free PMC Article
Cytologic characteristics of circulating epithelioid cells in pancreatic disease.
Rosenbaum MW, Cauley CE, Kulemann B, Liss AS, Castillo CF, Warshaw AL, Lillemoe KD, Thayer SP, Pitman MB
Cancer Cytopathol 2017 May;125(5):332-340. Epub 2017 Mar 3 doi: 10.1002/cncy.21841. PMID: 28257167Free PMC Article
Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
Arch Surg 2009 Jun;144(6):527-31. doi: 10.1001/archsurg.2009.80. PMID: 19528385

Recent systematic reviews

Neuroendocrine carcinoma of the minor papilla with pancreas divisum: a case report and review of the literature.
Saito K, Matsuo Y, Denda Y, Nonoyama K, Murase H, Kato T, Hayashi Y, Imafuji H, Morimoto M, Ogawa R, Takahashi H, Takiguchi S
J Med Case Rep 2023 Mar 28;17(1):127. doi: 10.1186/s13256-023-03828-x. PMID: 36973791Free PMC Article
Management and outcomes of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A systematic review and pooled analysis of 56 patients.
Ziogas IA, Rallis KS, Tasoudis PT, Moris D, Schulick RD, Del Chiaro M
Eur J Surg Oncol 2023 Apr;49(4):682-687. Epub 2023 Jan 9 doi: 10.1016/j.ejso.2023.01.005. PMID: 36646615
Somatostatinoma of the Ampulla of Vater: A Systematic Review.
Giannakodimos I, Giannakodimos A, Ziogou A, Frountzas M, Kritikos N, Vlachos K, Toutouzas KG, Schizas D
J Gastrointestin Liver Dis 2022 Dec 17;31(4):459-466. doi: 10.15403/jgld-4383. PMID: 36535044
The clinical profiles, management, and prognostic factors of biliary mixed neuroendocrine nonneuroendocrine neoplasms: A systematic review of the literature.
Wen LJ, Chen JH, Xu HJ, Yu Q, Deng Y, Liu K
Medicine (Baltimore) 2020 Dec 11;99(50):e23271. doi: 10.1097/MD.0000000000023271. PMID: 33327249Free PMC Article

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...