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Bile duct mucinous cystic neoplasm with an associated invasive carcinoma

MedGen UID:
137737
•Concept ID:
C0334286
•
Neoplastic Process
Synonyms: Bile Duct Cystadenocarcinoma; Bile duct cystadenocarcinoma; bile duct cystadenocarcinoma; bile duct cystadenocarcinoma (morphologic abnormality); Bile Duct MCN with an Associated Invasive Carcinoma; Bile Duct Mucinous Cystic Neoplasm with an Associated Invasive Carcinoma; bile duct mucinous cystic neoplasm with an associated invasive carcinoma; Biliary Cystadenocarcinoma; Cystadenocarcinoma of Bile Duct; cystadenocarcinoma of bile duct; Cystadenocarcinoma of the Bile Duct; cystadenocarcinoma of the bile duct
SNOMED CT: Bile duct cystadenocarcinoma (50422007)
 
Monarch Initiative: MONDO:0002868

Definition

A mucinous cystic neoplasm that arises from the intrahepatic or extrahepatic bile ducts and it is associated with an invasive carcinomatous component. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBile duct mucinous cystic neoplasm with an associated invasive carcinoma

Recent clinical studies

Etiology

Mucinous cystic neoplasms of the liver with biliary prolapse.
Kozaka K, Takahashi H, Inoue A, Graham RPD, Boyum JH, Heiken JP, Takahashi N
Jpn J Radiol 2023 Apr;41(4):409-416. Epub 2022 Nov 19 doi: 10.1007/s11604-022-01361-3. PMID: 36401061Free PMC Article
Invasive Intraductal Papillary Mucinous Neoplasms: CT Features of Colloid Carcinoma Versus Tubular Adenocarcinoma of the Pancreas.
Fouladi DF, Raman SP, Hruban RH, Fishman EK, Kawamoto S
AJR Am J Roentgenol 2020 May;214(5):1092-1100. Epub 2020 Mar 4 doi: 10.2214/AJR.19.21824. PMID: 32130045
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
Benign cystic neoplasm and endocrine tumours of the pancreas--when and how to operate--an overview.
Beger HG, Poch B, Vasilescu C
Int J Surg 2014;12(6):606-14. Epub 2014 Apr 15 doi: 10.1016/j.ijsu.2014.03.020. PMID: 24742543
Intraductal papillary neoplasm of the bile duct: a biliary equivalent to intraductal papillary mucinous neoplasm of the pancreas?
Rocha FG, Lee H, Katabi N, DeMatteo RP, Fong Y, D'Angelica MI, Allen PJ, Klimstra DS, Jarnagin WR
Hepatology 2012 Oct;56(4):1352-60. Epub 2012 Aug 27 doi: 10.1002/hep.25786. PMID: 22504729

Diagnosis

Bile duct dysplasia and associated invasive carcinoma: clinicopathological features, diagnosis, and practical challenges.
Liu Y, Yeh MM
Hum Pathol 2023 Feb;132:158-168. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.012. PMID: 35714833
Mucin-producing Cystic Hepatobiliary Neoplasms: Updated Nomenclature and Clinical, Pathologic, and Imaging Features.
Lee MH, Katabathina VS, Lubner MG, Shah HU, Prasad SR, Matkowskyj KA, Pickhardt PJ
Radiographics 2021 Oct;41(6):1592-1610. doi: 10.1148/rg.2021210011. PMID: 34597230Free PMC Article
Invasive Intraductal Papillary Mucinous Neoplasms: CT Features of Colloid Carcinoma Versus Tubular Adenocarcinoma of the Pancreas.
Fouladi DF, Raman SP, Hruban RH, Fishman EK, Kawamoto S
AJR Am J Roentgenol 2020 May;214(5):1092-1100. Epub 2020 Mar 4 doi: 10.2214/AJR.19.21824. PMID: 32130045
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
Intraductal papillary neoplasm of the bile duct: a biliary equivalent to intraductal papillary mucinous neoplasm of the pancreas?
Rocha FG, Lee H, Katabi N, DeMatteo RP, Fong Y, D'Angelica MI, Allen PJ, Klimstra DS, Jarnagin WR
Hepatology 2012 Oct;56(4):1352-60. Epub 2012 Aug 27 doi: 10.1002/hep.25786. PMID: 22504729

Therapy

Benign cystic neoplasm and endocrine tumours of the pancreas--when and how to operate--an overview.
Beger HG, Poch B, Vasilescu C
Int J Surg 2014;12(6):606-14. Epub 2014 Apr 15 doi: 10.1016/j.ijsu.2014.03.020. PMID: 24742543
Evaluation of surgical resection for pancreatic carcinoma at a Japanese single cancer institute.
Nanashima A, Tobinaga S, Abo T, Hatano K, Takeshita H, Nonaka T, Hidaka S, Tanaka K, Kunizaki M, Sawai T, Yasutake T, Nagayasu T
Hepatogastroenterology 2012 May;59(115):911-5. doi: 10.5754/hge10038. PMID: 22469741

Prognosis

MUC6 expression is a preferable prognostic marker for invasive mucinous adenocarcinoma of the lung.
Yamanoi K, Fujii C, Yuzuriha H, Kumazawa M, Shimoda M, Emoto K, Asamura H, Nakayama J
Histochem Cell Biol 2022 Jun;157(6):671-684. Epub 2022 Mar 30 doi: 10.1007/s00418-022-02093-1. PMID: 35353213
Mucin-producing Cystic Hepatobiliary Neoplasms: Updated Nomenclature and Clinical, Pathologic, and Imaging Features.
Lee MH, Katabathina VS, Lubner MG, Shah HU, Prasad SR, Matkowskyj KA, Pickhardt PJ
Radiographics 2021 Oct;41(6):1592-1610. doi: 10.1148/rg.2021210011. PMID: 34597230Free PMC Article
Invasive Intraductal Papillary Mucinous Neoplasms: CT Features of Colloid Carcinoma Versus Tubular Adenocarcinoma of the Pancreas.
Fouladi DF, Raman SP, Hruban RH, Fishman EK, Kawamoto S
AJR Am J Roentgenol 2020 May;214(5):1092-1100. Epub 2020 Mar 4 doi: 10.2214/AJR.19.21824. PMID: 32130045
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
Intraductal papillary neoplasm of the bile duct: a biliary equivalent to intraductal papillary mucinous neoplasm of the pancreas?
Rocha FG, Lee H, Katabi N, DeMatteo RP, Fong Y, D'Angelica MI, Allen PJ, Klimstra DS, Jarnagin WR
Hepatology 2012 Oct;56(4):1352-60. Epub 2012 Aug 27 doi: 10.1002/hep.25786. PMID: 22504729

Clinical prediction guides

MUC6 expression is a preferable prognostic marker for invasive mucinous adenocarcinoma of the lung.
Yamanoi K, Fujii C, Yuzuriha H, Kumazawa M, Shimoda M, Emoto K, Asamura H, Nakayama J
Histochem Cell Biol 2022 Jun;157(6):671-684. Epub 2022 Mar 30 doi: 10.1007/s00418-022-02093-1. PMID: 35353213
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
Benign cystic neoplasm and endocrine tumours of the pancreas--when and how to operate--an overview.
Beger HG, Poch B, Vasilescu C
Int J Surg 2014;12(6):606-14. Epub 2014 Apr 15 doi: 10.1016/j.ijsu.2014.03.020. PMID: 24742543
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
Intraductal papillary mucinous neoplasm of the pancreas: assessment of the likelihood of invasiveness with multisection CT.
Ogawa H, Itoh S, Ikeda M, Suzuki K, Naganawa S
Radiology 2008 Sep;248(3):876-86. Epub 2008 Jul 15 doi: 10.1148/radiol.2482071578. PMID: 18632526

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