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. 1991 Jan;213(1):21–25. doi: 10.1097/00000658-199101000-00004

Cholangiocarcinoma complicating primary sclerosing cholangitis.

C B Rosen 1, D M Nagorney 1, R H Wiesner 1, R J Coffey Jr 1, N F LaRusso 1
PMCID: PMC1358305  PMID: 1845927

Abstract

Cholangiocarcinoma is more likely to develop in patients with primary sclerosing cholangitis. Our aims were to describe the clinical presentation, course, and management of patients afflicted with both cholangiocarcinoma and primary sclerosing cholangitis and to estimate the prevalence of cholangiocarcinoma in patients with primary sclerosing cholangitis. A retrospective analysis was conducted of 30 patients with both primary sclerosing cholangitis and cholangiocarcinoma managed at our institution during an 8-year period. Development of cholangiocarcinoma was heralded by rapid clinical deterioration with jaundice, weight loss, and abdominal discomfort. Cholangiocarcinoma complicating primary sclerosing cholangitis often was detected at an advanced tumor stage, which precluded effective therapy, and overall median survival was 5 months. Earlier recognition and treatment of cholangiocarcinoma in such patients will be necessary to increase survival rates. Seventy patients with primary sclerosing cholangitis were followed prospectively in a clinical trial of medical therapy for an average of 30 months. Twelve patients died and five were found at autopsy to have cholangiocarcinoma. The potential for cholangiocarcinoma to develop in patients with primary sclerosing cholangitis may indicate that liver transplantation should be considered earlier in the course of the disease.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aadland E., Schrumpf E., Fausa O., Elgjo K., Heilo A., Aakhus T., Gjone E. Primary sclerosing cholangitis: a long-term follow-up study. Scand J Gastroenterol. 1987 Aug;22(6):655–664. doi: 10.3109/00365528709011139. [DOI] [PubMed] [Google Scholar]
  2. Akwari O. E., Van Heerden J. A., Foulk W. T., Baggenstoss A. H. Cancer of the bile ducts associated with ulcerative colitis. Ann Surg. 1975 Mar;181(3):303–309. doi: 10.1097/00000658-197503000-00010. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Belamaric J. Intrahepatic bile duct carcinoma and C. sinensis infection in Hong Kong. Cancer. 1973 Feb;31(2):468–473. doi: 10.1002/1097-0142(197302)31:2<468::aid-cncr2820310226>3.0.co;2-j. [DOI] [PubMed] [Google Scholar]
  4. Chapman R. W., Arborgh B. A., Rhodes J. M., Summerfield J. A., Dick R., Scheuer P. J., Sherlock S. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut. 1980 Oct;21(10):870–877. doi: 10.1136/gut.21.10.870. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. LaRusso N. F., Wiesner R. H., Ludwig J., MacCarty R. L., Beaver S. J., Zinsmeister A. R. Prospective trial of penicillamine in primary sclerosing cholangitis. Gastroenterology. 1988 Oct;95(4):1036–1042. doi: 10.1016/0016-5085(88)90180-1. [DOI] [PubMed] [Google Scholar]
  6. Ludwig J., Barham S. S., LaRusso N. F., Elveback L. R., Wiesner R. H., McCall J. T. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology. 1981 Nov-Dec;1(6):632–640. doi: 10.1002/hep.1840010612. [DOI] [PubMed] [Google Scholar]
  7. Marsh J. W., Jr, Iwatsuki S., Makowka L., Esquivel C. O., Gordon R. D., Todo S., Tzakis A., Miller C., Van Thiel D., Starzl T. E. Orthotopic liver transplantation for primary sclerosing cholangitis. Ann Surg. 1988 Jan;207(1):21–25. doi: 10.1097/00000658-198801000-00005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Mir-Madjlessi S. H., Farmer R. G., Sivak M. V., Jr Bile duct carcinoma in patients with ulcerative colitis. Relationship to sclerosing cholangitis: report of six cases and review of the literature. Dig Dis Sci. 1987 Feb;32(2):145–154. doi: 10.1007/BF01297102. [DOI] [PubMed] [Google Scholar]
  9. Ritchie J. K., Allan R. N., Macartney J., Thompson H., Hawley P. R., Cooke W. T. Biliary tract carcinoma associated with ulcerative colitis. Q J Med. 1974 Apr;43(170):263–279. [PubMed] [Google Scholar]
  10. TABET B. J. Primary carcinoma of the gallbladder. Report of forty-seven cases. Am J Surg. 1960 Sep;100:365–371. doi: 10.1016/0002-9610(60)90371-8. [DOI] [PubMed] [Google Scholar]
  11. Wee A., Ludwig J., Coffey R. J., Jr, LaRusso N. F., Wiesner R. H. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Pathol. 1985 Jul;16(7):719–726. doi: 10.1016/s0046-8177(85)80158-1. [DOI] [PubMed] [Google Scholar]
  12. Weinbren K., Mutum S. S. Pathological aspects of cholangiocarcinoma. J Pathol. 1983 Feb;139(2):217–238. doi: 10.1002/path.1711390210. [DOI] [PubMed] [Google Scholar]
  13. Wiesner R. H., LaRusso N. F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology. 1980 Aug;79(2):200–206. [PubMed] [Google Scholar]

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