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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

Dilated common bile duct is commonly associated with main duct Intraductal Papillary Mucinous Neoplasm of the pancreas.
Sbeit W, Shahin A, Khoury T
BMC Gastroenterol 2024 Jun 17;24(1):201. doi: 10.1186/s12876-024-03291-y. PMID: 38886637Free PMC Article
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
Malignant mediastinal lymphadenopathy detected by staging EUS in patients with pancreaticobiliary cancer.
Agarwal B, Gogia S, Eloubeidi MA, Correa AM, Ho L, Collins BT
Gastrointest Endosc 2005 Jun;61(7):849-53. doi: 10.1016/s0016-5107(05)00318-4. PMID: 15933686
Primary carcinoma of the gallbladder associated with chronic inflammatory bowel disease.
Joffe N, Antonioli DA
Clin Radiol 1981 May;32(3):319-24. doi: 10.1016/s0009-9260(81)80053-0. PMID: 6263537

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
Use of direct cholangiopancreatoscopy to identify pancreaticobiliary fistula.
Avila N, Tarnasky P, Kedia P
Endoscopy 2019 Dec;51(12):E380-E381. Epub 2019 Jul 4 doi: 10.1055/a-0929-3905. PMID: 31272114
"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
Biliary intraductal papillary mucinous tumor: diagnosis and localization by endoscopic retrograde cholangioscopy.
Somogyi L, Dimashkieh H, Weber FL Jr, Buell J
Gastrointest Endosc 2003 Apr;57(4):620-2. doi: 10.1067/mge.2003.157. PMID: 12665789
Endoscopic ultrasonography in the assessment of intra- and transmural infiltration of tumours in the oesophagus, stomach and papilla of Vater and in the detection of extraoesophageal lesions.
Tio TL, Tytgat GN
Endoscopy 1984 Nov;16(6):203-10. doi: 10.1055/s-2007-1018581. PMID: 6096116

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
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
DNA mismatch repair deficiency in ampullary carcinoma: a morphologic and immunohistochemical study of 54 cases.
Agaram NP, Shia J, Tang LH, Klimstra DS
Am J Clin Pathol 2010 May;133(5):772-80. doi: 10.1309/AJCPGDDE8PLLDRCC. PMID: 20395525
Malignant mediastinal lymphadenopathy detected by staging EUS in patients with pancreaticobiliary cancer.
Agarwal B, Gogia S, Eloubeidi MA, Correa AM, Ho L, Collins BT
Gastrointest Endosc 2005 Jun;61(7):849-53. doi: 10.1016/s0016-5107(05)00318-4. PMID: 15933686

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
"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
DNA mismatch repair deficiency in ampullary carcinoma: a morphologic and immunohistochemical study of 54 cases.
Agaram NP, Shia J, Tang LH, Klimstra DS
Am J Clin Pathol 2010 May;133(5):772-80. doi: 10.1309/AJCPGDDE8PLLDRCC. PMID: 20395525
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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