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Extrahepatic Bile Duct Mucinous Cystic Neoplasm with an Associated Invasive Carcinoma

MedGen UID:
474767
•Concept ID:
C3273134
•
Neoplastic Process
Synonyms: Extrahepatic Bile Duct Cystadenocarcinoma; Extrahepatic Bile Duct MCN with an Associated Invasive Carcinoma

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrahepatic Bile Duct Mucinous Cystic Neoplasm with an Associated Invasive Carcinoma

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
Histopathologic features and microsatellite instability of cancers of the papilla of vater and their precursor lesions.
Ruemmele P, Dietmaier W, Terracciano L, Tornillo L, Bataille F, Kaiser A, Wuensch PH, Heinmoeller E, Homayounfar K, Luettges J, Kloeppel G, Sessa F, Edmonston TB, Schneider-Stock R, Klinkhammer-Schalke M, Pauer A, Schick S, Hofstaedter F, Baumhoer D, Hartmann A
Am J Surg Pathol 2009 May;33(5):691-704. doi: 10.1097/PAS.0b013e3181983ef7. PMID: 19252434
Intraductal papillary-mucinous adenocarcinoma in the remnant pancreas after pancreatoduodenectomy for cancer of Vater's papilla associated with intraductal papillary-mucinous adenoma.
Ishida M, Egawa S, Sakata N, Mikami Y, Motoi F, Abe T, Fukuyama S, Sunamura M, Furukawa T, Unno M
J Hepatobiliary Pancreat Surg 2007;14(5):522-5. Epub 2007 Sep 28 doi: 10.1007/s00534-006-1199-9. PMID: 17909725
Expression and prognostic significance of 14-3-3sigma and ERM family protein expression in periampullary neoplasms.
Hustinx SR, Fukushima N, Zahurak ML, Riall TS, Maitra A, Brosens L, Cameron JL, Yeo CJ, Offerhaus GJ, Hruban RH, Goggins M
Cancer Biol Ther 2005 May;4(5):596-601. Epub 2005 May 13 doi: 10.4161/cbt.4.5.1748. PMID: 15908786
Carcinoma of the ampulla of Vater: comparative histologic/immunohistochemical classification and follow-up.
Zhou H, Schaefer N, Wolff M, Fischer HP
Am J Surg Pathol 2004 Jul;28(7):875-82. doi: 10.1097/00000478-200407000-00005. PMID: 15223956

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
Intraductal papillary-mucinous adenocarcinoma in the remnant pancreas after pancreatoduodenectomy for cancer of Vater's papilla associated with intraductal papillary-mucinous adenoma.
Ishida M, Egawa S, Sakata N, Mikami Y, Motoi F, Abe T, Fukuyama S, Sunamura M, Furukawa T, Unno M
J Hepatobiliary Pancreat Surg 2007;14(5):522-5. Epub 2007 Sep 28 doi: 10.1007/s00534-006-1199-9. PMID: 17909725
Expression and prognostic significance of 14-3-3sigma and ERM family protein expression in periampullary neoplasms.
Hustinx SR, Fukushima N, Zahurak ML, Riall TS, Maitra A, Brosens L, Cameron JL, Yeo CJ, Offerhaus GJ, Hruban RH, Goggins M
Cancer Biol Ther 2005 May;4(5):596-601. Epub 2005 May 13 doi: 10.4161/cbt.4.5.1748. PMID: 15908786
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
Telomerase activity in periampullary tumors correlates with aggressive malignancy.
Balcom JH 4th, Keck T, Warshaw AL, Antoniu B, Graeme-Cook F, Fernández-del Castillo C
Ann Surg 2001 Sep;234(3):344-50; discussion 350-1. doi: 10.1097/00000658-200109000-00008. PMID: 11524587Free PMC Article

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
Duodenum-preserving total pancreatic head resection for cystic neoplasm: a limited but cancer-preventive procedure.
Beger HG, Rau BM, Gansauge F, Schwarz M, Siech M, Poch B
Langenbecks Arch Surg 2008 Jul;393(4):589-98. Epub 2008 Apr 1 doi: 10.1007/s00423-008-0323-5. PMID: 18379818
Intraductal papillary-mucinous adenocarcinoma in the remnant pancreas after pancreatoduodenectomy for cancer of Vater's papilla associated with intraductal papillary-mucinous adenoma.
Ishida M, Egawa S, Sakata N, Mikami Y, Motoi F, Abe T, Fukuyama S, Sunamura M, Furukawa T, Unno M
J Hepatobiliary Pancreat Surg 2007;14(5):522-5. Epub 2007 Sep 28 doi: 10.1007/s00534-006-1199-9. PMID: 17909725
Expression and prognostic significance of 14-3-3sigma and ERM family protein expression in periampullary neoplasms.
Hustinx SR, Fukushima N, Zahurak ML, Riall TS, Maitra A, Brosens L, Cameron JL, Yeo CJ, Offerhaus GJ, Hruban RH, Goggins M
Cancer Biol Ther 2005 May;4(5):596-601. Epub 2005 May 13 doi: 10.4161/cbt.4.5.1748. PMID: 15908786
Carcinoma of the ampulla of Vater: comparative histologic/immunohistochemical classification and follow-up.
Zhou H, Schaefer N, Wolff M, Fischer HP
Am J Surg Pathol 2004 Jul;28(7):875-82. doi: 10.1097/00000478-200407000-00005. PMID: 15223956

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
A grading system can predict clinical and economic outcomes of pancreatic fistula after pancreaticoduodenectomy: results in 755 consecutive patients.
Daskalaki D, Butturini G, Molinari E, Crippa S, Pederzoli P, Bassi C
Langenbecks Arch Surg 2011 Jan;396(1):91-8. Epub 2010 Nov 3 doi: 10.1007/s00423-010-0719-x. PMID: 21046413
Duodenum-preserving total pancreatic head resection for cystic neoplasm: a limited but cancer-preventive procedure.
Beger HG, Rau BM, Gansauge F, Schwarz M, Siech M, Poch B
Langenbecks Arch Surg 2008 Jul;393(4):589-98. Epub 2008 Apr 1 doi: 10.1007/s00423-008-0323-5. PMID: 18379818
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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