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Ampulla of Vater Large Cell Neuroendocrine Carcinoma

MedGen UID:
474114
•Concept ID:
C3272481
•
Neoplastic Process
Synonyms: Ampulla of Vater Large Cell NEC; Large Cell 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. It is characterized by the presence of malignant large cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmpulla of Vater Large Cell Neuroendocrine Carcinoma

Professional guidelines

PubMed

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

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
Carcinoids and high-grade neuroendocrine carcinomas of the ampulla of vater: a comparative analysis of 139 cases from the surveillance, epidemiology, and end results program-a population based study.
Albores-Saavedra J, Hart A, Chablé-Montero F, Henson DE
Arch Pathol Lab Med 2010 Nov;134(11):1692-6. doi: 10.5858/2009-0697-OAR.1. PMID: 21043824

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
A Metastatic Neuroendocrine Eyelid Carcinoma Originating From the Ampulla of Vater.
Jung SK, Yang SW, Kyung Hong E, Seo Park W
Ophthalmic Plast Reconstr Surg 2020 Jul/Aug;36(4):e95-e96. doi: 10.1097/IOP.0000000000001609. PMID: 32049941
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
Neuroendocrine carcinoma of the ampulla of Vater: a clinicopathologic evaluation.
Selvakumar E, Rajendran S, Balachandar TG, Kannan DG, Jeswanth S, Ravichandran P, Surendran R
Hepatobiliary Pancreat Dis Int 2008 Aug;7(4):422-5. PMID: 18693180
Large cell neuroendocrine carcinoma of the ampulla of Vater with glandular differentiation.
Cheng SP, Yang TL, Chang KM, Liu CL
J Clin Pathol 2004 Oct;57(10):1098-100. doi: 10.1136/jcp.2004.019414. PMID: 15452169Free PMC Article

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
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
Neuroendocrine carcinoma of the ampulla of Vater: a clinicopathologic evaluation.
Selvakumar E, Rajendran S, Balachandar TG, Kannan DG, Jeswanth S, Ravichandran P, Surendran R
Hepatobiliary Pancreat Dis Int 2008 Aug;7(4):422-5. PMID: 18693180
Large cell neuroendocrine carcinoma of the ampulla of Vater with glandular differentiation.
Cheng SP, Yang TL, Chang KM, Liu CL
J Clin Pathol 2004 Oct;57(10):1098-100. doi: 10.1136/jcp.2004.019414. PMID: 15452169Free PMC Article
Large cell neuroendocrine carcinoma of the ampulla of vater.
Cavazza A, Gallo M, Valcavi R, De Marco L, Gardini G
Arch Pathol Lab Med 2003 Feb;127(2):221-3. doi: 10.5858/2003-127-221-LCNCOT. PMID: 12562240

Clinical prediction guides

Inhibin-expressing clear cell neuroendocrine tumor of the ampulla: an unusual presentation of von Hippel-Lindau disease.
Gucer H, Szentgyorgyi E, Ezzat S, Asa SL, Mete O
Virchows Arch 2013 Oct;463(4):593-7. Epub 2013 Aug 4 doi: 10.1007/s00428-013-1465-6. PMID: 23913169
Pathologic observations of the duodenum in 615 consecutive duodenal specimens in a single Japanese hospital: II. malignant lesions.
Terada T
Int J Clin Exp Pathol 2012;5(1):52-7. Epub 2012 Jan 1 PMID: 22295147Free PMC Article

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