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Mucobilia: current aspects in the management of a rare cause of malignant biliary obstruction

  • Topics
  • Current issues in mucin-producing tumor of the bile duct
  • Published:
Journal of Hepato-Biliary-Pancreatic Sciences

Abstract

Mucobilia is a rare pathologic condition characterized by the abnormal secretion and accumulation of abundant mucus within the biliary tree. It is usually seen in association with mucin-producing hepatobiliary and pancreatic tumors. Neoplastic transformation of these tumors can range from low-grade dysplasia to invasive adenocarcinoma. Mucobilia usually presents with signs and symptoms of biliary obstruction, which may span from jaundice to cholangitis with progression to septic complications in severe cases. Occurrence of hepatolithiasis has also been attributed to mucobilia, which raises the concern of an increased risk for the development of cholangiocarcinoma. Precise radiological evaluation and detailed histopathological tissue diagnosis followed by execution of appropriate surgical therapy is vital in the integrated management of mucin-producing biliary neoplasms. This review will address the etiologies and symptomatology of mucobilia as well as discuss current aspects in the management of mucobilia and its causative etiologies.

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References

  1. Chamberlain RS, Blumgart LH. Mucobilia in association with a biliary cystadenocarcinoma of the caudate duct: a rare cause of malignant biliary obstruction. HPB Surg. 2000;11:345–51.

    Article  CAS  PubMed  Google Scholar 

  2. Chen MF. Mucobilia: the clinical spectrum, diagnosis and treatment. J Gastroenterol Hepatol. 1998;13:1084–90.

    Article  CAS  PubMed  Google Scholar 

  3. Tsiftsis D, Christodoulakis M, de Bree E, Sanidas E. Primary intrahepatic biliary cystadenomatous tumors. J Surg Oncol. 1997;64:341–6.

    Article  CAS  PubMed  Google Scholar 

  4. Caroli J, Soupault R, Champaeu M. Papillomes et papillomatoses de la voie billiaire principale. Rev Med Chir Mal Foie. 1959;34:191–5.

    CAS  PubMed  Google Scholar 

  5. Goinard P, Pelissier G, Streit P. Mucinose des voies biliaries. Mem Acad Chir. 1960;86:849–54.

    CAS  PubMed  Google Scholar 

  6. Hadjis NS, Slater RN, Blumgart LH. Mucobilia: an unusual cause of jaundice. Br J Surg. 1987;74:48–9.

    Article  CAS  PubMed  Google Scholar 

  7. Tu JF, Zhu GB, Jiang FZ, Zhang QY. Diagnosis and treatment of mucobilia: report of 8 cases. Hepatobiliary Pancreat Dis Int. 2003;2:106–9.

    PubMed  Google Scholar 

  8. Kuo CM, Changchien CS, Wu KL, Chuah SK, Chiu KW, Chiu YC, et al. Mucin-producing cholangiocarcinoma: clinical experience of 24 cases in 16 years. Scand J Gastroenterol. 2005;40:455–9.

    Article  PubMed  Google Scholar 

  9. Läuffer JM, Baer HU, Maurer CA, Stoupis C, Zimmerman A, Büchler MW. Biliary cystadenoma of the liver: the need for complete resection. Eur J Cancer. 1998;34:1845–51.

    Article  PubMed  Google Scholar 

  10. Styne P, Warren GH, Kumpe DA, Halgrimson C, Kern F Jr. Obstructive cholangitis secondary to mucus secreted by a solitary papillary bile duct tumor. Gastroenterology. 1986;90:748–53.

    CAS  PubMed  Google Scholar 

  11. Kokubo T, Itai Y, Ohtomo K, Itoh K, Kawauchi N, Minami M. Mucin-hypersecreting intrahepatic biliary neoplasms. Radiology. 1988;168:609–14.

    CAS  PubMed  Google Scholar 

  12. Jan YY, Chen MF, Hung CF. Cholangiography of mucin-hypersecreting intrahepatic biliary neoplasms. J Hepatobiliary Pancreat Surg. 1977;4:180–4.

    Article  Google Scholar 

  13. Jan YY, Chen MF, Chen TJ. Cholangiocarcinoma with mucobilia. J Formous Med Assoc. 1994;93:S149–55.

    Google Scholar 

  14. Iemoto Y, Kondo Y, Fukamachi S. Biliary cystadenocarcinoma with peritoneal carcinomatosis. Cancer. 1981;48:1664–7.

    Article  CAS  PubMed  Google Scholar 

  15. Takayasu K, Muramatsu Y, Moriyama N, Yamada T, Hasegawa H, Hirohashi S, et al. Imaging diagnosis of the bile duct of cystadenocarcinoma. Cancer. 1988;61:941–6.

    Article  CAS  PubMed  Google Scholar 

  16. Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature. Cancer. 1977;39:322–38.

    Article  CAS  PubMed  Google Scholar 

  17. Rambaud S, Nores JM, Meeus F, Paolaggi JA. Malignant papillomatosis of the bile ducts: a new indication for liver transplantation? Am J Gastroenterol. 1989;84:448–9.

    CAS  PubMed  Google Scholar 

  18. Yeh TS, Tseng JH, Chiu CT, Liu NJ, Chen TC, Jan YY, et al. Cholangiographic spectrum of intraductal papillary mucinous neoplasm of the bile ducts. Ann Surg. 2006;244:248–53.

    Article  PubMed  Google Scholar 

  19. Yamashita Y, Fukuzawa K, Taketomi A, Aishima S, Yoshizumi T, Uchiyama H, et al. Mucin-hypersecreting bile duct neoplasm characterized by clinicopathological resemblance to intraductal papillary mucinous neoplasm (IPMN) of the pancreas. World J Surg Oncol. 2007;5:98.

    Article  PubMed  Google Scholar 

  20. Lim JH, Yoon KH, Kim SH, Kim HY, Lim HK, Song SY, et al. Intraductal papillary mucinous tumor of the bile ducts. Radiographics. 2004;24:53–66.

    Article  PubMed  Google Scholar 

  21. Lee SS, Kim MH, Lee SK, Jang SJ, Song MH, Kim KP, et al. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer. 2004;100:783–93.

    Article  PubMed  Google Scholar 

  22. Chen MF, Jan YY, Wang CS, Hwang TL, Jeng LB, Chen SC, et al. A reappraisal of cholangiocarcinoma in patient with hepatolithiasis. Cancer. 1993;71:2461–5.

    Article  CAS  PubMed  Google Scholar 

  23. Shimonishi T, Zen Y, Chen TC, Chen MF, Jan YY, Yeh TS, et al. Increasing expression of gastrointestinal phenotypes and p53 along with histologic progression of intraductal papillary neoplasia of the liver. Hum Pathol. 2002;33:503–11.

    Article  CAS  PubMed  Google Scholar 

  24. Capizzi PJ, Rosen CB, Nagorney DM. Intermittent jaundice by tumor emboli from intrahepatic cholangiocarcinoma. Gastroenterology. 1992;103:1669–73.

    CAS  PubMed  Google Scholar 

  25. Tsou YK, Liu NJ, Wu RC, Lee CS, Tang JH, Hung CF, et al. Endoscopic retrograde cholangiography in the diagnosis and treatment of mucobilia. Scand J Gastroenterol. 2008;43:1137–44.

    Article  PubMed  Google Scholar 

  26. Kubo S, Kinoshita H, Hirohashi K, Hamba H. Hepatolithiasis associated with cholangiocarcinoma. World J Surg. 1995;19:637–41.

    Article  CAS  PubMed  Google Scholar 

  27. Zen Y, Fujii T, Itatsu K, Nakamura K, Minato H, Kasashima S, et al. Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas. Hepatology. 2006;44:1333–43.

    Article  CAS  PubMed  Google Scholar 

  28. Wolf HK, Garcia JA, Bossen EH. Oncocytic differentiation in intrahepatic biliary cystadenocarcinoma. Mod Pathol. 1992;5:665–8.

    CAS  PubMed  Google Scholar 

  29. Adsay NV, Adair CF, Heffess CS, Klimstra DS. Intraductal oncocytic papillary neoplasms of the pancreas. Am J Surg Pathol. 1996;20:980–4.

    Article  CAS  PubMed  Google Scholar 

  30. Sakamoto E, Hayakawa N, Kamiya J, Kondo S, Nagino M, Kanai M, et al. Treatment strategy for mucin-producing intrahepatic cholangiocarcinoma: value of percutaneous transhepatic biliary drainage and cholangioscopy. World J Surg. 1999;23:1038–43.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Ronald Scott Chamberlain.

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Brown, MA., Sakpal, S.V. & Chamberlain, R.S. Mucobilia: current aspects in the management of a rare cause of malignant biliary obstruction. J Hepatobiliary Pancreat Sci 17, 205–210 (2010). https://doi.org/10.1007/s00534-009-0150-2

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  • DOI: https://doi.org/10.1007/s00534-009-0150-2

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