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Bile Duct Mucinous Cystic Neoplasm, High Grade

MedGen UID:
474704
•Concept ID:
C3273071
•
Neoplastic Process
Synonyms: Bile Duct MCN with High Grade Intraepithelial Neoplasia; Bile Duct Mucinous Cystic Neoplasm with High Grade Intraepithelial Neoplasia; Bile Duct Mucinous Cystic Neoplasm, High-Grade

Definition

A non-invasive mucinous cystic neoplasm that arises from the intrahepatic or extrahepatic bile ducts. It is characterized by the presence of severe atypia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBile Duct Mucinous Cystic Neoplasm, High Grade

Recent clinical studies

Etiology

The CT fish mouth ampulla sign: a highly specific finding in main duct and mixed intraductal papillary mucinous neoplasms.
Zhang TT, Sadler TJ, Whitley S, Brais R, Godfrey E
Br J Radiol 2019 Nov;92(1103):20190461. Epub 2019 Aug 28 doi: 10.1259/bjr.20190461. PMID: 31430199Free PMC Article
Isolated Pancreatic Uncinate Duct IPMN.
Maker AV, Maker VK
J Gastrointest Surg 2017 Apr;21(4):744-745. Epub 2017 Feb 15 doi: 10.1007/s11605-017-3380-7. PMID: 28205123
Cystadenomas of the liver and extrahepatic bile ducts: Morphologic and immunohistochemical characterization of the biliary and intestinal variants.
Albores-Saavedra J, Córdova-Ramón JC, Chablé-Montero F, Dorantes-Heredia R, Henson DE
Ann Diagn Pathol 2015 Jun;19(3):124-9. Epub 2015 Mar 6 doi: 10.1016/j.anndiagpath.2015.03.001. PMID: 25792461
Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association.
Kubota K, Nakanuma Y, Kondo F, Hachiya H, Miyazaki M, Nagino M, Yamamoto M, Isayama H, Tabata M, Kinoshita H, Kamisawa T, Inui K
J Hepatobiliary Pancreat Sci 2014 Mar;21(3):176-85. Epub 2013 Aug 1 doi: 10.1002/jhbp.23. PMID: 23908126
The clinicopathological spectrum and management of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B).
D'souza MA, Isaksson B, Löhr M, Enochsson L, Swahn F, Lundell L, Arnelo U
Scand J Gastroenterol 2013 Apr;48(4):473-9. Epub 2013 Jan 21 doi: 10.3109/00365521.2012.722672. PMID: 23330596

Diagnosis

The CT fish mouth ampulla sign: a highly specific finding in main duct and mixed intraductal papillary mucinous neoplasms.
Zhang TT, Sadler TJ, Whitley S, Brais R, Godfrey E
Br J Radiol 2019 Nov;92(1103):20190461. Epub 2019 Aug 28 doi: 10.1259/bjr.20190461. PMID: 31430199Free PMC Article
Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.
Kunovsky L, Kala Z, Svaton R, Moravcik P, Mazanec J, Husty J, Prochazka V
Ann Hepatol 2018 May-June;17(3):519-524. Epub 2018 Apr 9 doi: 10.5604/01.3001.0011.7397. PMID: 29735801
The Evolving Role of Pathology in New Developments, Classification, Terminology, and Diagnosis of Pancreatobiliary Neoplasms.
Reid MD, Lewis MM, Willingham FF, Adsay NV
Arch Pathol Lab Med 2017 Mar;141(3):366-380. Epub 2017 Jan 5 doi: 10.5858/arpa.2016-0262-SA. PMID: 28055239
Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas.
Matsumoto Y, Miyamoto H, Fukuya A, Nakamura F, Goji T, Kitamura S, Kimura T, Okamoto K, Sogabe M, Muguruma N, Shimada M, Bando Y, Takayama T
Clin J Gastroenterol 2017 Apr;10(2):185-190. Epub 2017 Jan 4 doi: 10.1007/s12328-016-0711-2. PMID: 28054178
The clinicopathological spectrum and management of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B).
D'souza MA, Isaksson B, Löhr M, Enochsson L, Swahn F, Lundell L, Arnelo U
Scand J Gastroenterol 2013 Apr;48(4):473-9. Epub 2013 Jan 21 doi: 10.3109/00365521.2012.722672. PMID: 23330596

Prognosis

The CT fish mouth ampulla sign: a highly specific finding in main duct and mixed intraductal papillary mucinous neoplasms.
Zhang TT, Sadler TJ, Whitley S, Brais R, Godfrey E
Br J Radiol 2019 Nov;92(1103):20190461. Epub 2019 Aug 28 doi: 10.1259/bjr.20190461. PMID: 31430199Free PMC Article
Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.
Kunovsky L, Kala Z, Svaton R, Moravcik P, Mazanec J, Husty J, Prochazka V
Ann Hepatol 2018 May-June;17(3):519-524. Epub 2018 Apr 9 doi: 10.5604/01.3001.0011.7397. PMID: 29735801
Cystadenomas of the liver and extrahepatic bile ducts: Morphologic and immunohistochemical characterization of the biliary and intestinal variants.
Albores-Saavedra J, Córdova-Ramón JC, Chablé-Montero F, Dorantes-Heredia R, Henson DE
Ann Diagn Pathol 2015 Jun;19(3):124-9. Epub 2015 Mar 6 doi: 10.1016/j.anndiagpath.2015.03.001. PMID: 25792461
Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association.
Kubota K, Nakanuma Y, Kondo F, Hachiya H, Miyazaki M, Nagino M, Yamamoto M, Isayama H, Tabata M, Kinoshita H, Kamisawa T, Inui K
J Hepatobiliary Pancreat Sci 2014 Mar;21(3):176-85. Epub 2013 Aug 1 doi: 10.1002/jhbp.23. PMID: 23908126
The clinicopathological spectrum and management of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B).
D'souza MA, Isaksson B, Löhr M, Enochsson L, Swahn F, Lundell L, Arnelo U
Scand J Gastroenterol 2013 Apr;48(4):473-9. Epub 2013 Jan 21 doi: 10.3109/00365521.2012.722672. PMID: 23330596

Clinical prediction guides

The CT fish mouth ampulla sign: a highly specific finding in main duct and mixed intraductal papillary mucinous neoplasms.
Zhang TT, Sadler TJ, Whitley S, Brais R, Godfrey E
Br J Radiol 2019 Nov;92(1103):20190461. Epub 2019 Aug 28 doi: 10.1259/bjr.20190461. PMID: 31430199Free PMC Article
Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.
Kunovsky L, Kala Z, Svaton R, Moravcik P, Mazanec J, Husty J, Prochazka V
Ann Hepatol 2018 May-June;17(3):519-524. Epub 2018 Apr 9 doi: 10.5604/01.3001.0011.7397. PMID: 29735801
Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas.
Matsumoto Y, Miyamoto H, Fukuya A, Nakamura F, Goji T, Kitamura S, Kimura T, Okamoto K, Sogabe M, Muguruma N, Shimada M, Bando Y, Takayama T
Clin J Gastroenterol 2017 Apr;10(2):185-190. Epub 2017 Jan 4 doi: 10.1007/s12328-016-0711-2. PMID: 28054178
Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association.
Kubota K, Nakanuma Y, Kondo F, Hachiya H, Miyazaki M, Nagino M, Yamamoto M, Isayama H, Tabata M, Kinoshita H, Kamisawa T, Inui K
J Hepatobiliary Pancreat Sci 2014 Mar;21(3):176-85. Epub 2013 Aug 1 doi: 10.1002/jhbp.23. PMID: 23908126
Histologic and imaging features of mural nodules in mucinous pancreatic cysts.
Zhong N, Zhang L, Takahashi N, Shalmiyev V, Canto MI, Clain JE, Deutsch JC, DeWitt J, Eloubeidi MA, Gleeson FC, Levy MJ, Mallery S, Raimondo M, Rajan E, Stevens T, Topazian M
Clin Gastroenterol Hepatol 2012 Feb;10(2):192-8, 198.e1-2. Epub 2011 Oct 5 doi: 10.1016/j.cgh.2011.09.029. PMID: 21982970

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