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Cholangiocarcinoma
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==Prognosis== Surgical resection offers the only potential chance of cure in cholangiocarcinoma. For non-resectable cases, the five-year survival rate is 0% where the disease is inoperable because distal lymph nodes show metastases,<ref>{{cite journal | vauthors = Yamamoto M, Takasaki K, Yoshikawa T | title = Lymph node metastasis in intrahepatic cholangiocarcinoma | journal = Japanese Journal of Clinical Oncology | volume = 29 | issue = 3 | pages = 147β50 | date = March 1999 | pmid = 10225697 | doi = 10.1093/jjco/29.3.147 | doi-access = free }}</ref> and less than 5% in general.<ref>{{cite journal | vauthors = Farley DR, Weaver AL, Nagorney DM | title = 'Natural history' of unresected cholangiocarcinoma: patient outcome after noncurative intervention | journal = Mayo Clinic Proceedings | volume = 70 | issue = 5 | pages = 425β9 | date = May 1995 | pmid = 7537346 | doi = 10.4065/70.5.425 }}</ref> Overall mean duration of survival is less than 6 months in people with metastatic disease.<ref>{{cite journal | vauthors = Grove MK, Hermann RE, Vogt DP, Broughan TA | title = Role of radiation after operative palliation in cancer of the proximal bile ducts | journal = American Journal of Surgery | volume = 161 | issue = 4 | pages = 454β8 | date = April 1991 | pmid = 1709795 | doi = 10.1016/0002-9610(91)91111-U }}</ref> For surgical cases, 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 surgically with a [[Whipple procedure]]; long-term survival rates range from 15 to 25%, although one series reported a [[five-year survival rate|five-year survival]] of 54% for people with no involvement of the [[lymph node]]s.<ref>Studies of surgical outcomes in distal cholangiocarcinoma include: * {{cite journal | vauthors = Nakeeb A, Pitt HA, Sohn TA, Coleman J, Abrams RA, Piantadosi S, Hruban RH, Lillemoe KD, Yeo CJ, Cameron JL | display-authors = 6 | title = Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors | journal = Annals of Surgery | volume = 224 | issue = 4 | pages = 463β73; discussion 473β5 | date = October 1996 | pmid = 8857851 | pmc = 1235406 | doi = 10.1097/00000658-199610000-00005 }} * {{cite journal | vauthors = Jang JY, Kim SW, Park DJ, Ahn YJ, Yoon YS, Choi MG, Suh KS, Lee KU, Park YH | display-authors = 6 | title = Actual long-term outcome of extrahepatic bile duct cancer after surgical resection | journal = Annals of Surgery | volume = 241 | issue = 1 | pages = 77β84 | date = January 2005 | pmid = 15621994 | pmc = 1356849 | doi = 10.1097/01.sla.0000150166.94732.88 }} * {{cite journal | vauthors = Bortolasi L, Burgart LJ, Tsiotos GG, Luque-De LeΓ³n E, Sarr MG | title = Adenocarcinoma of the distal bile duct. A clinicopathologic outcome analysis after curative resection | journal = Digestive Surgery | volume = 17 | issue = 1 | pages = 36β41 | year = 2000 | pmid = 10720830 | doi = 10.1159/000018798 | s2cid = 23190342 }} * {{cite journal | vauthors = Fong Y, Blumgart LH, Lin E, Fortner JG, Brennan MF | title = Outcome of treatment for distal bile duct cancer | journal = British Journal of Surgery | volume = 83 | issue = 12 | pages = 1712β5 | date = December 1996 | pmid = 9038548 | doi = 10.1002/bjs.1800831217 | s2cid = 30172073 }}</ref> Intrahepatic cholangiocarcinomas (those arising from the bile ducts within the [[liver]]) are usually treated with [[hepatectomy|partial hepatectomy]]. Various series have reported survival estimates after surgery ranging from 22 to 66%; the outcome may depend on involvement of lymph nodes and completeness of the surgery.<ref>Studies of outcome in intrahepatic cholangiocarcinoma include: * {{cite journal | vauthors = Nakeeb A, Pitt HA, Sohn TA, Coleman J, Abrams RA, Piantadosi S, Hruban RH, Lillemoe KD, Yeo CJ, Cameron JL | display-authors = 6 | title = Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors | journal = Annals of Surgery | volume = 224 | issue = 4 | pages = 463β73; discussion 473β5 | date = October 1996 | pmid = 8857851 | pmc = 1235406 | doi = 10.1097/00000658-199610000-00005 }} * {{cite journal | vauthors = Lieser MJ, Barry MK, Rowland C, Ilstrup DM, Nagorney DM | title = Surgical management of intrahepatic cholangiocarcinoma: a 31-year experience | journal = Journal of Hepato-Biliary-Pancreatic Surgery | volume = 5 | issue = 1 | pages = 41β7 | year = 1998 | pmid = 9683753 | doi = 10.1007/PL00009949 }} * {{cite journal | vauthors = Valverde A, Bonhomme N, Farges O, Sauvanet A, Flejou JF, Belghiti J | title = Resection of intrahepatic cholangiocarcinoma: a Western experience | journal = Journal of Hepato-Biliary-Pancreatic Surgery | volume = 6 | issue = 2 | pages = 122β7 | year = 1999 | pmid = 10398898 | doi = 10.1007/s005340050094 }} * {{cite journal | vauthors = Nakagohri T, Asano T, Kinoshita H, Kenmochi T, Urashima T, Miura F, Ochiai T | display-authors = 6 | title = Aggressive surgical resection for hilar-invasive and peripheral intrahepatic cholangiocarcinoma | journal = World Journal of Surgery | volume = 27 | issue = 3 | pages = 289β93 | date = March 2003 | pmid = 12607053 | doi = 10.1007/s00268-002-6696-7 | s2cid = 25358444 }} * {{cite journal | vauthors = Weber SM, Jarnagin WR, Klimstra D, DeMatteo RP, Fong Y, Blumgart LH | title = Intrahepatic cholangiocarcinoma: resectability, recurrence pattern, and outcomes | journal = Journal of the American College of Surgeons | volume = 193 | issue = 4 | pages = 384β91 | date = October 2001 | pmid = 11584966 | doi = 10.1016/S1072-7515(01)01016-X }}</ref> 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 [[cholecystectomy|removal of the gallbladder]] and potentially part of the liver. In patients with operable perihilar tumors, reported 5-year survival rates range from 20 to 50%.<ref>Estimates of survival after surgery for perihilar cholangiocarcinoma include: * {{cite journal | vauthors = Burke EC, Jarnagin WR, Hochwald SN, Pisters PW, Fong Y, Blumgart LH | title = Hilar Cholangiocarcinoma: patterns of spread, the importance of hepatic resection for curative operation, and a presurgical clinical staging system | journal = Annals of Surgery | volume = 228 | issue = 3 | pages = 385β94 | date = September 1998 | pmid = 9742921 | pmc = 1191497 | doi = 10.1097/00000658-199809000-00011 }} * {{cite journal | vauthors = Tsao JI, Nimura Y, Kamiya J, Hayakawa N, Kondo S, Nagino M, Miyachi M, Kanai M, Uesaka K, Oda K, Rossi RL, Braasch JW, Dugan JM | display-authors = 6 | title = Management of hilar cholangiocarcinoma: comparison of an American and a Japanese experience | journal = Annals of Surgery | volume = 232 | issue = 2 | pages = 166β74 | date = August 2000 | pmid = 10903592 | pmc = 1421125 | doi = 10.1097/00000658-200008000-00003 }} * {{cite journal | vauthors = Chamberlain RS, Blumgart LH | title = Hilar cholangiocarcinoma: a review and commentary | journal = Annals of Surgical Oncology | volume = 7 | issue = 1 | pages = 55β66 | year = 2000 | pmid = 10674450 | doi = 10.1007/s10434-000-0055-4 | s2cid = 19569428 }} * {{cite journal | vauthors = Washburn WK, Lewis WD, Jenkins RL | title = Aggressive surgical resection for cholangiocarcinoma | journal = Archives of Surgery | volume = 130 | issue = 3 | pages = 270β6 | date = March 1995 | pmid = 7534059 | doi = 10.1001/archsurg.1995.01430030040006 }} * {{cite journal | vauthors = Nagino M, Nimura Y, Kamiya J, Kanai M, Uesaka K, Hayakawa N, Yamamoto H, Kondo S, Nishio H | display-authors = 6 | title = Segmental liver resections for hilar cholangiocarcinoma | journal = Hepato-Gastroenterology | volume = 45 | issue = 19 | pages = 7β13 | year = 1998 | pmid = 9496478 }} * {{cite journal | vauthors = Rea DJ, Munoz-Juarez M, Farnell MB, Donohue JH, Que FG, Crownhart B, Larson D, Nagorney DM | display-authors = 6 | title = Major hepatic resection for hilar cholangiocarcinoma: analysis of 46 patients | journal = Archives of Surgery | volume = 139 | issue = 5 | pages = 514β23; discussion 523β5 | date = May 2004 | pmid = 15136352 | doi = 10.1001/archsurg.139.5.514 | doi-access = free }} * {{cite journal | vauthors = Launois B, Reding R, Lebeau G, Buard JL | title = Surgery for hilar cholangiocarcinoma: French experience in a collective survey of 552 extrahepatic bile duct cancers | journal = Journal of Hepato-Biliary-Pancreatic Surgery | volume = 7 | issue = 2 | pages = 128β34 | year = 2000 | pmid = 10982604 | doi = 10.1007/s005340050166 }}</ref> The [[prognosis]] may be worse for people with primary sclerosing cholangitis who develop cholangiocarcinoma, likely because the cancer is not detected until it is advanced.<ref name="autopsy"/><ref>{{cite journal | vauthors = Kaya M, de Groen PC, Angulo P, Nagorney DM, Gunderson LL, Gores GJ, Haddock MG, Lindor KD | display-authors = 6 | title = Treatment of cholangiocarcinoma complicating primary sclerosing cholangitis: the Mayo Clinic experience | journal = American Journal of Gastroenterology | volume = 96 | issue = 4 | pages = 1164β9 | date = April 2001 | doi = 10.1111/j.1572-0241.2001.03696.x | pmid = 11316165 | s2cid = 295347 }}</ref> Some evidence suggests that outcomes may be improving with more aggressive surgical approaches and [[adjuvant therapy]].<ref>{{cite journal | vauthors = Nakeeb A, Tran KQ, Black MJ, Erickson BA, Ritch PS, Quebbeman EJ, Wilson SD, Demeure MJ, Rilling WS, Dua KS, Pitt HA | display-authors = 6 | title = Improved survival in resected biliary malignancies | journal = Surgery | volume = 132 | issue = 4 | pages = 555β63; discission 563β4 | date = October 2002 | pmid = 12407338 | doi = 10.1067/msy.2002.127555 }}</ref>
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