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Extrahepatic Bile Duct Large Cell Neuroendocrine Carcinoma

MedGen UID:
473537
•Concept ID:
C2111716
•
Neoplastic Process
Synonym: Extrahepatic Bile Duct Large Cell NEC

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the extrahepatic bile ducts. It is characterized by the presence of malignant large cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrahepatic Bile Duct 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
From Krukenberg to today: the ever present problems posed by metastatic tumors in the ovary. Part II.
Young RH
Adv Anat Pathol 2007 May;14(3):149-77. doi: 10.1097/PAP.0b013e3180504abf. PMID: 17452813

Diagnosis

Pseudoprogression of lung large cell neuroendocrine carcinoma resembling pancreatic cancer during durvalumab therapy.
Hattori A, Nishikawa K, Ito K, Tachikawa N, Fujibe K, Tanaka S, Oiwa M, Hataji O
Clin J Gastroenterol 2022 Jun;15(3):668-672. Epub 2022 Jan 4 doi: 10.1007/s12328-021-01588-z. PMID: 34984635
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
Neuroendocrine carcinoma diagnosis from bile duct cytological specimens: A retrospective single-center study.
Ishida M, Okano K, Sandoh K, Ito H, Ikeura T, Mitsuyama T, Miyoshi H, Shimatani M, Takaoka M, Okazaki K, Tsuta K
Diagn Cytopathol 2020 Feb;48(2):154-158. Epub 2019 Nov 7 doi: 10.1002/dc.24334. PMID: 31697402
Large Cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct.
Park JY, Jeon TJ
Korean J Gastroenterol 2018 Dec 25;72(6):318-321. doi: 10.4166/kjg.2018.72.6.318. PMID: 30642152
Neuroendocrine carcinoma of the extrahepatic bile duct: case report and literature review.
Sasatomi E, Nalesnik MA, Marsh JW
World J Gastroenterol 2013 Jul 28;19(28):4616-23. doi: 10.3748/wjg.v19.i28.4616. PMID: 23901240Free PMC Article

Therapy

Large-cell neuroendocrine carcinoma of the bile duct: Case report of surgical treatment and adjuvant chemotherapy of 2 cases.
Seo CH, Choi HJ
Medicine (Baltimore) 2024 Sep 27;103(39):e39848. doi: 10.1097/MD.0000000000039848. PMID: 39331932Free 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
Mixed adenoneuroendocrine carcinoma of the extrahepatic bile duct: a case report.
Zhang HW, Kou K, Qi J, Xie EB, Wang M, Li Y, Lv GY, Wang GY
J Int Med Res 2019 Jul;47(7):3421-3426. Epub 2019 Jun 3 doi: 10.1177/0300060519850391. PMID: 31154920Free PMC Article
Neuroendocrine carcinoma of the extrahepatic bile duct: A case report.
Oshiro Y, Gen R, Hashimoto S, Oda T, Sato T, Ohkohchi N
World J Gastroenterol 2016 Aug 14;22(30):6960-4. doi: 10.3748/wjg.v22.i30.6960. PMID: 27570432Free PMC Article
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
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

Clinical prediction guides

Pseudoprogression of lung large cell neuroendocrine carcinoma resembling pancreatic cancer during durvalumab therapy.
Hattori A, Nishikawa K, Ito K, Tachikawa N, Fujibe K, Tanaka S, Oiwa M, Hataji O
Clin J Gastroenterol 2022 Jun;15(3):668-672. Epub 2022 Jan 4 doi: 10.1007/s12328-021-01588-z. PMID: 34984635
Neuroendocrine carcinoma of the common bile duct associated with congenital bile duct dilatation: a case report.
Kiya Y, Nagakawa Y, Takishita C, Osakabe H, Nishino H, Akashi M, Yamaguchi H, Nagao T, Oono R, Katsumata K, Tsuchida A
BMC Gastroenterol 2021 Jun 12;21(1):257. doi: 10.1186/s12876-021-01777-7. PMID: 34118881Free PMC Article
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
Mucin-producing gallbladder adenocarcinoma with focal small cell and large cell neuroendocrine differentiation associated with pancreaticobiliary maljunction.
Oshiro H, Matsuo K, Mawatari H, Inayama Y, Yamanaka S, Nagahama K, Endo I, Shimada H, Nakajima A, Kubota K
Pathol Int 2008 Dec;58(12):780-6. doi: 10.1111/j.1440-1827.2008.02311.x. PMID: 19067853

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