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Extrahepatic bile duct mucinous adenocarcinoma

MedGen UID:
167830
•Concept ID:
C0861856
•
Neoplastic Process
Synonyms: Bile Duct Colloid Carcinoma; bile duct colloid carcinoma; Bile Duct Colloidal Carcinoma; bile duct colloidal carcinoma; Bile Duct Mucinous Adenocarcinoma; bile duct mucinous adenocarcinoma; Colloid Adenocarcinoma of Bile Duct; colloid adenocarcinoma of bile duct; Colloid Adenocarcinoma of Extrahepatic Bile Duct; colloid adenocarcinoma of extrahepatic bile duct; Colloid Adenocarcinoma of the Bile Duct; colloid adenocarcinoma of the bile duct; Colloid Adenocarcinoma of the Extrahepatic Bile Duct; colloid adenocarcinoma of the extrahepatic bile duct; Colloid Bile Duct Adenocarcinoma; colloid bile duct adenocarcinoma; Colloid Bile Duct Carcinoma; colloid bile duct carcinoma; Colloid Carcinoma of Bile Duct; colloid carcinoma of bile duct; Colloid Carcinoma of the Bile Duct; colloid carcinoma of the bile duct; Colloid Extrahepatic Bile Duct Adenocarcinoma; colloid extrahepatic bile duct adenocarcinoma; Colloidal Adenocarcinoma of Bile Duct; colloidal adenocarcinoma of bile duct; Colloidal Adenocarcinoma of Extrahepatic Bile Duct; colloidal adenocarcinoma of extrahepatic bile duct; Colloidal Adenocarcinoma of the Bile Duct; colloidal adenocarcinoma of the bile duct; Colloidal Adenocarcinoma of the Extrahepatic Bile Duct; colloidal adenocarcinoma of the extrahepatic bile duct; Colloidal Bile Duct Adenocarcinoma; colloidal bile duct adenocarcinoma; Colloidal Bile Duct Carcinoma; colloidal bile duct carcinoma; Colloidal Carcinoma of Bile Duct; colloidal carcinoma of bile duct; Colloidal Carcinoma of the Bile Duct; colloidal carcinoma of the bile duct; Colloidal Extrahepatic Bile Duct Adenocarcinoma; colloidal extrahepatic bile duct adenocarcinoma; Extrahepatic Bile Duct Mucinous Adenocarcinoma; extrahepatic bile duct mucinous adenocarcinoma; Mucinous Adenocarcinoma of Bile Duct; mucinous adenocarcinoma of bile duct; Mucinous Adenocarcinoma of Extrahepatic Bile Duct; mucinous adenocarcinoma of extrahepatic bile duct; Mucinous Adenocarcinoma of the Bile Duct; mucinous adenocarcinoma of the bile duct; Mucinous Adenocarcinoma of the Extrahepatic Bile Duct; mucinous adenocarcinoma of the extrahepatic bile duct; Mucinous Bile Duct Adenocarcinoma; mucinous bile duct adenocarcinoma; Mucinous Carcinoma of Bile Duct; mucinous carcinoma of bile duct; Mucinous Carcinoma of the Bile Duct; mucinous carcinoma of the bile duct; Mucinous Extrahepatic Bile Duct Adenocarcinoma; mucinous extrahepatic bile duct adenocarcinoma
 
Monarch Initiative: MONDO:0002739

Definition

An adenocarcinoma that arises from the extrahepatic bile ducts. It is characterized by the presence of extracellular mucin that constitutes more than fifty-percent of the tumor. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrahepatic bile duct mucinous adenocarcinoma

Professional guidelines

PubMed

A statement by the Japan-Korea expert pathologists for future clinicopathological and molecular analyses toward consensus building of intraductal papillary neoplasm of the bile duct through several opinions at the present stage.
Nakanuma Y, Jang KT, Fukushima N, Furukawa T, Hong SM, Kim H, Lee KB, Zen Y, Jang JY, Kubota K
J Hepatobiliary Pancreat Sci 2018 Mar;25(3):181-187. Epub 2018 Feb 12 doi: 10.1002/jhbp.532. PMID: 29272078
CD56 expression aids in the differential diagnosis of cholangiocarcinomas and benign cholangiocellular lesions.
Gütgemann I, Haas S, Berg JP, Zhou H, Büttner R, Fischer HP
Virchows Arch 2006 Apr;448(4):407-11. Epub 2006 Jan 13 doi: 10.1007/s00428-005-0145-6. PMID: 16411132
Radiologic spectrum of cholangiocarcinoma: emphasis on unusual manifestations and differential diagnoses.
Lee WJ, Lim HK, Jang KM, Kim SH, Lee SJ, Lim JH, Choo IW
Radiographics 2001 Oct;21 Spec No:S97-S116. doi: 10.1148/radiographics.21.suppl_1.g01oc12s97. PMID: 11598251

Recent clinical studies

Etiology

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
Intraductal papillary neoplasm of the bile duct: Radiologic findings in a new disease.
Rodríguez Vargas D, Parada Blázquez MJ, Vargas Serrano B
Radiologia (Engl Ed) 2020 Jan-Feb;62(1):28-37. Epub 2019 Oct 9 doi: 10.1016/j.rx.2019.07.003. PMID: 31606127
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
Endoscopic diagnosis of intraductal papillary mucinous neoplasm of the bile duct.
Tsuyuguchi T, Sakai Y, Sugiyama H, Miyakawa K, Ishihara T, Ohtsuka M, Miyazaki M, Yokosuka O
J Hepatobiliary Pancreat Sci 2010 May;17(3):230-5. Epub 2009 Aug 11 doi: 10.1007/s00534-009-0153-z. PMID: 19669677
Carcinoma of extrahepatic bile ducts and gallbladder metastatic to the ovary: a report of 16 cases.
Khunamornpong S, Lerwill MF, Siriaunkgul S, Suprasert P, Pojchamarnwiputh S, Chiangmai WN, Young RH
Int J Gynecol Pathol 2008 Jul;27(3):366-79. doi: 10.1097/PGP.0b013e31815d6903. PMID: 18580314

Diagnosis

Intraductal papillary neoplasm of the bile duct: Radiologic findings in a new disease.
Rodríguez Vargas D, Parada Blázquez MJ, Vargas Serrano B
Radiologia (Engl Ed) 2020 Jan-Feb;62(1):28-37. Epub 2019 Oct 9 doi: 10.1016/j.rx.2019.07.003. PMID: 31606127
Role of Immunohistochemistry in the Subtyping of Periampullary Adenocarcinoma.
Bakshi N, Dhawan S, Nundy S, Rao S, Chopra P, Bhalla S
Int J Surg Pathol 2019 Sep;27(6):598-608. Epub 2019 Apr 3 doi: 10.1177/1066896919837606. PMID: 30942099
A statement by the Japan-Korea expert pathologists for future clinicopathological and molecular analyses toward consensus building of intraductal papillary neoplasm of the bile duct through several opinions at the present stage.
Nakanuma Y, Jang KT, Fukushima N, Furukawa T, Hong SM, Kim H, Lee KB, Zen Y, Jang JY, Kubota K
J Hepatobiliary Pancreat Sci 2018 Mar;25(3):181-187. Epub 2018 Feb 12 doi: 10.1002/jhbp.532. PMID: 29272078
"Biliary Diseases with Pancreatic Counterparts": Cross-sectional Imaging Findings.
Katabathina VS, Flaherty EM, Dasyam AK, Menias CO, Riddle ND, Lath N, Kozaka K, Matsui O, Nakanuma Y, Prasad SR
Radiographics 2016 Mar-Apr;36(2):374-92. Epub 2016 Feb 1 doi: 10.1148/rg.2016150071. PMID: 26824512
Endoscopic diagnosis of intraductal papillary mucinous neoplasm of the bile duct.
Tsuyuguchi T, Sakai Y, Sugiyama H, Miyakawa K, Ishihara T, Ohtsuka M, Miyazaki M, Yokosuka O
J Hepatobiliary Pancreat Sci 2010 May;17(3):230-5. Epub 2009 Aug 11 doi: 10.1007/s00534-009-0153-z. PMID: 19669677

Therapy

Mucin-filled CBD, difficult to manage cholangitis.
Kumar M, Sonika U, Sachdeva S, Dalal A
BMJ Case Rep 2021 Jan 11;14(1) doi: 10.1136/bcr-2020-238363. PMID: 33431462Free PMC Article
Long-term survival after hepatectomy for hepatic recurrence of carcinoma of the papilla of Vater.
Kitami CE, Kurosaki I, Koyama Y, Makino H, Hatakeyama K
J Hepatobiliary Pancreat Surg 2005;12(4):321-3. doi: 10.1007/s00534-005-0972-5. PMID: 16133701
Pylorus-preserving total pancreatectomy. Early and late results.
Wagner M, Z'graggen K, Vagianos CE, Redaelli CA, Holzinger F, Sadowski C, Kulli C, Zimmermann H, Baer HU, Büchler MW
Dig Surg 2001;18(3):188-95. doi: 10.1159/000050128. PMID: 11464008
Periampullary cancer. A clinicopathologic study of sixty-two patients.
Wise L, Pizzimbono C, Dehner LP
Am J Surg 1976 Feb;131(2):141-8. doi: 10.1016/0002-9610(76)90087-8. PMID: 56140

Prognosis

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
Intraductal papillary neoplasm of the bile duct: Radiologic findings in a new disease.
Rodríguez Vargas D, Parada Blázquez MJ, Vargas Serrano B
Radiologia (Engl Ed) 2020 Jan-Feb;62(1):28-37. Epub 2019 Oct 9 doi: 10.1016/j.rx.2019.07.003. PMID: 31606127
Role of Immunohistochemistry in the Subtyping of Periampullary Adenocarcinoma.
Bakshi N, Dhawan S, Nundy S, Rao S, Chopra P, Bhalla S
Int J Surg Pathol 2019 Sep;27(6):598-608. Epub 2019 Apr 3 doi: 10.1177/1066896919837606. PMID: 30942099
Precancerous lesions of the biliary tree.
Klöppel G, Adsay V, Konukiewitz B, Kleeff J, Schlitter AM, Esposito I
Best Pract Res Clin Gastroenterol 2013 Apr;27(2):285-97. doi: 10.1016/j.bpg.2013.04.002. PMID: 23809246
Liver, gallbladder, extrahepatic bile ducts, and pancreas.
Carriaga MT, Henson DE
Cancer 1995 Jan 1;75(1 Suppl):171-90. doi: 10.1002/1097-0142(19950101)75:1+<171::aid-cncr2820751306>3.0.co;2-2. PMID: 8000995

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
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
"Biliary Diseases with Pancreatic Counterparts": Cross-sectional Imaging Findings.
Katabathina VS, Flaherty EM, Dasyam AK, Menias CO, Riddle ND, Lath N, Kozaka K, Matsui O, Nakanuma Y, Prasad SR
Radiographics 2016 Mar-Apr;36(2):374-92. Epub 2016 Feb 1 doi: 10.1148/rg.2016150071. PMID: 26824512
The utility of immunohistochemistry in subtyping adenocarcinoma of the ampulla of vater.
Ang DC, Shia J, Tang LH, Katabi N, Klimstra DS
Am J Surg Pathol 2014 Oct;38(10):1371-9. doi: 10.1097/PAS.0000000000000230. PMID: 24832159
Carcinoma of extrahepatic bile ducts and gallbladder metastatic to the ovary: a report of 16 cases.
Khunamornpong S, Lerwill MF, Siriaunkgul S, Suprasert P, Pojchamarnwiputh S, Chiangmai WN, Young RH
Int J Gynecol Pathol 2008 Jul;27(3):366-79. doi: 10.1097/PGP.0b013e31815d6903. PMID: 18580314

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