Cholangiocarcinoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Prognosis

Surgical resection offers the only potential chance of cure in cholangiocarcinoma. The odds of cure vary depending on the tumor location and whether the tumor can be completely, or only partially, removed.

Distal cholangiocarcinomas (those arising from the common bile duct) are generally treated with a Whipple procedure; long-term survival rates range from 15%–25%, although one series reported a five year survival of 54% for patients with no involvement of the lymph nodes.[1] Intrahepatic cholangiocarcinomas (those arising from the bile ducts within the liver) are usually treated with partial hepatectomy. Various series have reported survival estimates after surgery ranging from 22%–66%; the outcome may depend on involvement of lymph nodes and completeness of the surgery.[2] Perihilar cholangiocarcinomas (those occurring near where the bile ducts exit the liver) are least likely to be operable. When surgery is possible, they are generally treated with an aggressive approach often including removal of the gallbladder and potentially part of the liver. In patients with operable perihilar tumors, reported 5-year survival rates range from 20%–50%.[3]

The prognosis may be worse for patients with primary sclerosing cholangitis who develop cholangiocarcinoma, likely because the cancer is not detected until it is advanced.[4][5] Some evidence suggests that outcomes may be improving with more aggressive surgical approaches and adjuvant therapy.[6]

Complications

  • Infection
  • Liver failure
  • Spread (metastasis) of tumor to other organs

References

  1. Studies of surgical outcomes in distal cholangiocarcinoma include:
    • Nakeeb A, Pitt H, Sohn T, Coleman J, Abrams R, Piantadosi S, Hruban R, Lillemoe K, Yeo C, Cameron J (1996). "Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors". Ann Surg. 224 (4): 463–73, discussion 473-5. PMID 8857851.
    • Nagorney D, Donohue J, Farnell M, Schleck C, Ilstrup D (1993). "Outcomes after curative resections of cholangiocarcinoma". Arch Surg. 128 (8): 871–7, discussion 877-9. PMID 8393652.
    • Jang J, Kim S, Park D, Ahn Y, Yoon Y, Choi M, Suh K, Lee K, Park Y (2005). "Actual long-term outcome of extrahepatic bile duct cancer after surgical resection". Ann Surg. 241 (1): 77–84. PMID 15621994.
    • Bortolasi L, Burgart L, Tsiotos G, Luque-De León E, Sarr M (2000). "Adenocarcinoma of the distal bile duct. A clinicopathologic outcome analysis after curative resection". Dig Surg. 17 (1): 36–41. PMID 10720830.
    • Fong Y, Blumgart L, Lin E, Fortner J, Brennan M (1996). "Outcome of treatment for distal bile duct cancer". Br J Surg. 83 (12): 1712–5. PMID 9038548.
  2. Studies of outcome in intrahepatic cholangiocarcinoma include:
    • Nakeeb A, Pitt H, Sohn T, Coleman J, Abrams R, Piantadosi S, Hruban R, Lillemoe K, Yeo C, Cameron J (1996). "Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors". Ann Surg. 224 (4): 463–73, discussion 473-5. PMID 8857851.
    • Lieser M, Barry M, Rowland C, Ilstrup D, Nagorney D (1998). "Surgical management of intrahepatic cholangiocarcinoma: a 31-year experience". J Hepatobiliary Pancreat Surg. 5 (1): 41–7. PMID 9683753.
    • Valverde A, Bonhomme N, Farges O, Sauvanet A, Flejou J, Belghiti J (1999). "Resection of intrahepatic cholangiocarcinoma: a Western experience". J Hepatobiliary Pancreat Surg. 6 (2): 122–7. PMID 10398898.
    • Nakagohri T, Asano T, Kinoshita H, Kenmochi T, Urashima T, Miura F, Ochiai T (2003). "Aggressive surgical resection for hilar-invasive and peripheral intrahepatic cholangiocarcinoma". World J Surg. 27 (3): 289–93. PMID 12607053.
    • Weber S, Jarnagin W, Klimstra D, DeMatteo R, Fong Y, Blumgart L (2001). "Intrahepatic cholangiocarcinoma: resectability, recurrence pattern, and outcomes". J Am Coll Surg. 193 (4): 384–91. PMID 11584966.
  3. Estimates of survival after surgery for perihilar cholangiocarcinoma include:
    • Burke E, Jarnagin W, Hochwald S, Pisters P, Fong Y, Blumgart L (1998). "Hilar Cholangiocarcinoma: patterns of spread, the importance of hepatic resection for curative operation, and a presurgical clinical staging system". Ann Surg. 228 (3): 385–94. PMID 9742921.
    • Tsao J, Nimura Y, Kamiya J, Hayakawa N, Kondo S, Nagino M, Miyachi M, Kanai M, Uesaka K, Oda K, Rossi R, Braasch J, Dugan J (2000). "Management of hilar cholangiocarcinoma: comparison of an American and a Japanese experience". Ann Surg. 232 (2): 166–74. PMID 10903592.
    • Chamberlain R, Blumgart L. "Hilar cholangiocarcinoma: a review and commentary". Ann Surg Oncol. 7 (1): 55–66. PMID 10674450.
    • Washburn W, Lewis W, Jenkins R (1995). "Aggressive surgical resection for cholangiocarcinoma". Arch Surg. 130 (3): 270–6. PMID 7534059.
    • Nagino M, Nimura Y, Kamiya J, Kanai M, Uesaka K, Hayakawa N, Yamamoto H, Kondo S, Nishio H. "Segmental liver resections for hilar cholangiocarcinoma". Hepatogastroenterology. 45 (19): 7–13. PMID 9496478.
    • Rea D, Munoz-Juarez M, Farnell M, Donohue J, Que F, Crownhart B, Larson D, Nagorney D (2004). "Major hepatic resection for hilar cholangiocarcinoma: analysis of 46 patients". Arch Surg. 139 (5): 514–23, discussion 523-5. PMID 15136352.
    • Launois B, Reding R, Lebeau G, Buard J (2000). "Surgery for hilar cholangiocarcinoma: French experience in a collective survey of 552 extrahepatic bile duct cancers". J Hepatobiliary Pancreat Surg. 7 (2): 128–34. PMID 10982604.
  4. Kaya M, de Groen P, Angulo P, Nagorney D, Gunderson L, Gores G, Haddock M, Lindor K (2001). "Treatment of cholangiocarcinoma complicating primary sclerosing cholangitis: the Mayo Clinic experience". Am J Gastroenterol. 96 (4): 1164–9. PMID 11316165.
  5. Nakeeb A, Tran K, Black M, Erickson B, Ritch P, Quebbeman E, Wilson S, Demeure M, Rilling W, Dua K, Pitt H (2002). "Improved survival in resected biliary malignancies". Surgery. 132 (4): 555–63, discission 563-4. PMID 12407338.

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