Cholangiocarcinoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

Common complications of cholangiocarcinoma include infection, liver failure, and tumor metastasis. Prognosis is generally poor, and the survival rate of patients with cholangiocarcinoma mainly depends on extent of the tumor and resectability. Even with resection, prognosis is poor with 5-year survival of only 10-44%.^[1] The presence of primary sclerosing cholangitis is associated with a particularly poor prognosis among patients with cholangiocarcinoma.

Complications

Common complications of cholangiocarcinoma include:

Prognosis

Depending on the extent of the cholangiocarcinoma at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor with 5-year survival of only 10-44%.
The prognosis may be worse for patients with primary sclerosing cholangitis who develop cholangiocarcinoma.
The most important factor in prognosis of cholangiocarcinoma is whether or not the tumor is able to be resected.

Extent of the tumor

Patients with multiple tumors, larger tumors and tumors that have spread to nearby blood vessels or lymph nodes have a poor outcome.

Resectability=

Tumors that can be completely removed by surgery (resectable) have a more favorable prognosis than tumors that cannot be removed by surgery (unresectable).^[2]

Distal cholangiocarcinoma: Long-term survival rates range from 15%–25%. ^[3]
Intrahepatic cholangiocarcinoma: Survival estimates after surgery ranging from 22%–66%.^[4]
Perihilar cholangiocarcinoma: 5 years survival rates range from 20%–50%.^[5]

Surgical margins

The best prognostic factors are resection of tumor-free surgical margin without lymph node invasion.
Tumor diameter, histology, and differentiation are poor predictors of good outcome with 5-year survival rates varying from 20 to 60%.^[2]
For extrahepatic cholangiocarcinoma, 5 year survival rate is approximately 30% after resection of tumor-free surgical margins.Majority of patients have recurrence due to following reasons:
- Disseminated tumors
- Formation of new tumors in previously oncogenic liver tissue

References

↑ Cholangiocarcinoma. Radiopaedia. http://radiopaedia.org/articles/cholangiocarcinoma
↑ ^2.0 ^2.1 Macias, Rocio I. R. (2014). "Cholangiocarcinoma: Biology, Clinical Management, and Pharmacological Perspectives". ISRN Hepatology. 2014: 1–13. doi:10.1155/2014/828074. ISSN 2314-4041.
↑
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.
↑
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.
↑
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.

Template:WH Template:WS

[radio-1] Cholangiocarcinoma. Radiopaedia. http://radiopaedia.org/articles/cholangiocarcinoma

[Macias2014-2] 2.0 ^2.1 Macias, Rocio I. R. (2014). "Cholangiocarcinoma: Biology, Clinical Management, and Pharmacological Perspectives". ISRN Hepatology. 2014: 1–13. doi:10.1155/2014/828074. ISSN 2314-4041.

[3] 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.

[4] 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.

[5] 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.

[6] 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.

[7] 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.

[8] 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.

[4] 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.

[10] 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.

[11] 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.

[12] 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.

[13] 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.

[14] 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.

[5] 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.

[16] 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.

[17] 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.

[18] Chamberlain R, Blumgart L. "Hilar cholangiocarcinoma: a review and commentary". Ann Surg Oncol. 7 (1): 55–66. PMID 10674450.

[19] Washburn W, Lewis W, Jenkins R (1995). "Aggressive surgical resection for cholangiocarcinoma". Arch Surg. 130 (3): 270–6. PMID 7534059.

[20] 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.

[21] 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.

[22] 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.

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