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Bile Duct Mucinous Cystic Neoplasm, Low Grade

MedGen UID:
474702
•Concept ID:
C3273069
•
Neoplastic Process
Synonyms: Bile Duct MCN with Low Grade Intraepithelial Neoplasia; Bile Duct Mucinous Cystic Neoplasm with Low Grade Intraepithelial Neoplasia; Bile Duct Mucinous Cystic Neoplasm, Low-Grade

Definition

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

Term Hierarchy

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

Recent clinical studies

Etiology

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
Characterization of intraductal papillary neoplasm of bile duct with respect to histopathologic similarities to pancreatic intraductal papillary mucinous neoplasm.
Nakanuma Y, Kakuda Y, Uesaka K, Miyata T, Yamamoto Y, Fukumura Y, Sato Y, Sasaki M, Harada K, Takase M
Hum Pathol 2016 May;51:103-13. Epub 2016 Jan 19 doi: 10.1016/j.humpath.2015.12.022. PMID: 27067788
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

Mucinous cystic neoplasm of the liver with biliary communication: case report and surgical therapeutic option.
Ferreira R, Abreu P, Jeismann VB, Segatelli V, Coelho FF, David AI
BMC Surg 2020 Dec 11;20(1):328. doi: 10.1186/s12893-020-01003-3. PMID: 33308210Free PMC Article
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
Mucinous cystic neoplasm of the liver with low grade dysplasia of the liver.
Pirdopska T, Terziev I, Taneva I, Dimitrova V
Khirurgiia (Sofiia) 2014;(3):51-2. PMID: 25799624
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

Prognosis

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
Cystic tumor of the liver without ovarian-like stroma or bile duct communication: two case reports and a review of the literature.
Kishida N, Shinoda M, Masugi Y, Itano O, Fujii-Nishimura Y, Ueno A, Kitago M, Hibi T, Abe Y, Yagi H, Tanimoto A, Tanabe M, Sakamaoto M, Kitagawa Y
World J Surg Oncol 2014 Jul 21;12:229. doi: 10.1186/1477-7819-12-229. PMID: 25047921Free PMC Article
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
Outcome of duodenum-preserving resection of the head of the pancreas for intraductal papillary-mucinous neoplasm.
Hirano S, Kondo S, Ambo Y, Tanaka E, Morikawa T, Okushiba S, Katoh H
Dig Surg 2004;21(3):242-5. Epub 2004 Jun 30 doi: 10.1159/000079494. PMID: 15237258

Clinical prediction guides

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
Outcome of duodenum-preserving resection of the head of the pancreas for intraductal papillary-mucinous neoplasm.
Hirano S, Kondo S, Ambo Y, Tanaka E, Morikawa T, Okushiba S, Katoh H
Dig Surg 2004;21(3):242-5. Epub 2004 Jun 30 doi: 10.1159/000079494. PMID: 15237258
Almost all infiltrating colloid carcinomas of the pancreas and periampullary region arise from in situ papillary neoplasms: a study of 39 cases.
Seidel G, Zahurak M, Iacobuzio-Donahue C, Sohn TA, Adsay NV, Yeo CJ, Lillemoe KD, Cameron JL, Hruban RH, Wilentz RE
Am J Surg Pathol 2002 Jan;26(1):56-63. doi: 10.1097/00000478-200201000-00006. PMID: 11756769

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