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The First Affiliated Hospital, Zhejiang University School of Medicine

Biliary T umor. Xu Xiao M.D. Ph.D. The First Affiliated Hospital, Zhejiang University School of Medicine The Key Laboratory of Combined Multi-Organ Transplantation Ministry of Public Health Hangzhou, China. The Biliary Anatomy. C ystic T riangle. 胆囊三角（ cystic triangle ）

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The First Affiliated Hospital, Zhejiang University School of Medicine

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  1. Biliary Tumor Xu Xiao M.D. Ph.D. The First Affiliated Hospital, Zhejiang University School of Medicine The Key Laboratory of Combined Multi-Organ Transplantation Ministry of Public Health Hangzhou, China

  2. The Biliary Anatomy

  3. Cystic Triangle 胆囊三角（cystic triangle） 肝总管、肝下缘和胆囊管围的三角区 胆囊动脉、肝右动脉、胆囊淋巴结及副右肝管在胆囊三角经过 肝总管 胆囊管

  4. Physiology • Bile Ducts • Intrahepatic biliary tract • Extrahepatic biliary tract • Gallbladder • Concentrates and stores hepatic bile during the fasting state and delivers bile into the duodenum in response to a meal • The gallbladder epithelial cell secretes at least two important products into the gallbladder lumen: glycoproteins and hydrogen ions • Sphincter of Oddi • It creates a high-pressure zone between the bile duct and the duodenum • The sphincter regulates the flow of bile and pancreatic juice into the duodenum, prevents the regurgitation of duodenal contents into the biliary tract

  5. Frequently used Assistant Examination ERCP CT cholangiogram shows enhanced imaging of the biliary system comparable to MRC. Intrahepatic and extrahepatic biliary ducts are clearly seen in this patient. PTCD

  6. Malignant Biliary Disease • Gallbladder Cancer • Bile Duct Cancer • Metastatic and Other Tumors

  7. Gallbladder Cancer

  8. Gallbladder Cancer • An aggressive malignancy that occurs predominantly in elderly people. • Besides the exceptional cases detected incidentally at the time of cholecystectomy for gallstone disease, which are usually early stage, the prognosis for most patients is poor. • Reported 5-year survival rates:5% ~38%.

  9. Gallbladder Cancer • Incidence • Cancer of the gallbladder is two to three times more common in women than men, in part because of the higher incidence of gallstones in women. • More than 75% of patients with this malignancy are older than 65 years. • The incidence of gallbladder cancer varies considerably with both ethnic background and geographic location.

  10. Risk Factors Gallbladder Cancer • Gallstones • Calcified gallbladder (porcelain) • Biliary Salmonella typhi infection • Biliary adenomas

  11. Symptoms Gallbladder Cancer • Same as gallstone disease • Recurrent RUQ pain • Radiating to interscapular area • Nausea • Vomiting • Fatty food intolerance

  12. Gallbladder Cancer-Nevein staging • Stage I: intramucosal only; • (癌组织仅限于粘膜内，即原位癌) • Stage II:involvement of mucosa and muscularis; • (侵及肌层) • Stage III:involvement of all three layers; • (癌组织侵及胆囊壁全层) • Stage IV:involvement of all three layers and the cystic lymph node; • (侵及胆囊壁全层合并周围淋巴结转移) • Stage V:involvement of liver by direct extension or metastases, or metastases to any other organ • (直接侵及肝脏或转移至其他脏器或远处转移) JE Nevin, TJ Moran, S Kay, R King. Cancer, 1976

  13. Gallbladder Cancer-TNM staging Edge SB, et al. AJCC cancer staging handbook: from the AJCC cancer staging manual. 7th ed. New York: 2010

  14. Gallbladder Cancer • Diagnosis • Ultrasonography is often the first diagnostic modality used in the evaluation of patients with right upper quadrant abdominal pain. • A heterogeneous mass replacing the gallbladder lumen and an irregular gallbladder wall are common sonographic features of gallbladder cancer. • CT scan usually demonstrates a mass replacing the gallbladder or extending into adjacent organs. • Cholangiography also may be helpful in diagnosing jaundiced patients with gallbladder cancer. • The typical cholangiographic finding in gallbladder cancer is a long stricture of the common hepatic duct.

  15. Gallbladder Cancer

  16. Gallbladder Cancer • Management • The appropriate operative procedure for the patient with localized gallbladder cancer is determined by the pathologic stage. • Cancer of the gallbladder with invasion beyond (stages II and III) the gallbladder muscularis is associated with an increased incidence of regional lymph node metastases and should be managed with an “extended cholecystectomy.” • This includes lymphadenectomy of the cystic duct, pericholedochal, portal, right celiac, and posterior pancreatoduodenal lymph nodes. • The results of chemotherapy in the treatment of patients with gallbladder cancer have been quite poor.

  17. Gallbladder Cancer

  18. Gallbladder Cancer • Survival Improved survival due to an aggressive approach to gallbladder cancer comparing two time periods (TPs), 1990-1996 and 1996-2002 (circles) (P < .03).   (From Dixon E, Vollmer CM, Sahajpal A, et al: An aggressive surgical approach leads to improved survival in patients with gallbladder cancer: A 12-year study at a North American Center. Ann Surg 241:385-394, 2005.)

  19. Gallbladder Cancer • Survival Survival following surgical resection for T2 gallbladder cancer. Patients undergoing radical resection (triangles) are compared with patients undergoing simple cholecystectomy (circles) .   (From Fong Y, Jarnigan W, Blumgart LH: Gallbladder cancer: Comparison of patients presenting initially for definitive operation with those presenting after prior noncurative intervention. Ann Surg 232:557-569, 2000.)

  20. Cholangiocarcinoma is an adenocarcinoma of the intrahepatic or extrahepatic bile duct. Bile Duct Cancer Definition :

  21. Bile Duct Cancer • Incidence: • 1.0 per 100,000 per year • Male to female ration of 1.3:1 • Average age of presentation is 50-70 • Etiology: • Common features of risk factors include biliary stasis, bile duct stones, and infection • Choledocal cysts, hepatolithiasis • Other risk factors include liver flukes, nitrosoamines, dioxin exposure

  22. Bile Duct Cancer • Pathology • Over 95% of bile duct cancers are adenocarcinomas • Morphologically they are divided into nodular, scirrhous, diffusely infiltrating, or papillary • Anatomically they are divided into distal, proximal or perihilar tumors • About 2/3 are perihilar, and are referred to as Klatskin tumors and broken down according to the Bismuth Corlette classification

  23. Bile Duct Cancer Intrahepatic CCs: develop in the smaller bile duct branches inside the liver(肝内胆管癌) Hilar CCs: develop at the hilum (肝门部胆管癌) Extrahepatic CCs: originate in the bile duct along the hepato-duodenal ligament(肝外胆管癌) Murad Aljiffry,, et al. World J Gastroenterol, 2009

  24. Bismuth Classification for Klatskin tumors Tumor confined to the common hepatic duct I II Involve the common hepatic duct bifurcation Affect hepatic duct bifurcation and right hepatic duct IIIa IIIb Affect hepatic duct bifurcation and left hepatic duct Affect biliary confluence with right and left hepatic ducts IVa IVa+ multifocal distribution IVb Henri Bismuth, Ann Surg, 1992

  25. Bile Duct Cancer • Clinical Presentation More than 90% of patients with perihilar or distal tumors present with jaundice. Patients with intrahepatic cholangiocarcinoma are rarely jaundiced until late in the course of the disease. Less common presenting clinical features include pruritus, fever, mild abdominal pain, fatigue, anorexia, and weight loss. Cholangitis is not a frequent presenting finding but most commonly develops after biliary manipulation. Except for jaundice, the physical examination is usually normal in patients with cholangiocarcinoma.

  26. Bile Duct Cancer • Classification and Staging Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage II T2 N0 M0 Stage III T1 or T2 N1 or N2 M0 Stage IVA T3 Any N M0 Stage IVB Any T Any N M1 Tis, carcinoma in situ; T1, tumor invades the subepithelial connective tissue; T2, tumor invades peri. bromuscular connective tissue; T3, tumor invades adjacent organs. N0, no regional lymph node metastases; N1, metastasis to hepatoduodenal ligament lymph nodes; N2, metastasis to peripancreatic, periduodenal, periportal, celiac, and/or superior mesenteric artery lymph nodes. M0, no distant metastasis; M1, distant metastasis Adapted from Greene F, Page D, Fleming I, et al (eds): AJCC Cancer Staging Manual, 7th ed. New York, Springer-Verlag, 2010.

  27. Diagnosis Bile Duct Cancer • Tumor markers : CEA, CA 19-9 • Radiographic studies :Transabdominal ultrasound, CT, MRCP • Cholangiography : ERCP or PTC • Endoscopic ultrasound • PET • Angiography (rarely used)

  28. Bile Duct Cancer Computed tomography scan visualizes mass at hepatic duct bifurcation (arrow) resulting in bilateral biliary dilation and extensive perihilar malignancy

  29. Bile Duct Cancer ERCP

  30. Bile Duct Cancer MRCP MRCP

  31. Differential Diagnosis Bile Duct Cancer • Choledocholithiasis • Benign bile duct strictures (usually postoperative), • Sclerosing cholangitis • Compression of the CBD (secondary to chronic pancreatitis or pancreatic cancer)

  32. Bile Duct Cancer • Surgical excision is the only potential curative treatment. • Most tumors are unresectable and may require surgery or stenting for palliation in jaundiced individuals. • Intrahepatic tumors may be treated like HCC with appropriate liver resection. • Extrahepatic tumors may be treated with a Whipple Procedure. • Treatment

  33. Bile Duct Cancer

  34. Bile Duct Cancer • Prognosis • Unresectable disease has a survival of 5-8 months on average. • The overall 5-year survival for patients with resectable perihilar CA is 10-30%, and 40% with negative margins. • The op. mortality in perihilar disease is 6-8% • Distal disease has a mildly improved prognosis compared with perihilar disease. • Overall 5 year survival for resectible disease is 30-50%.

  35. Case 1 Female, 60y,Cholangiocarcinoma received liver resection on May 27th, 2010. Portal Vein Reconstruction MRCP before Operation

  36. Case 2 Femal, 54y, hilar Cholangiocarcinomareceived left hepatectomy +caudate resection+portal vein reconctruction Bismuth IIIb Portal vein invasion left hepatectomy +caudate resection+portal vein reconctruction Portal vein resection After portal vein reconstruction

  37. Femal, 50y, hilar Cholangiocarcinomareceived central hepatectomy Tumor Bismuth IV 胆道整形后 biliary reconstruction of Cholangiocarcinoma

  38. Liver Transplantation (LT)for Cholangiocarcinoma in Our Center Male, 57y, Cholangiocarcinoma , received liver transplantation on October 25th, 2005 before LT 5 years post LT

  39. LT for Cholangiocarcinoma in Our Center Wang Xiaoping, Male, 51y, Cholangiocarcinoma, received LT in 1999, Survival: 11 years Lin Hanbin, Male, 46y, Cholangiocarcinoma, received LT in 2000, Survival: 10 years

  40. LT for Cholangiocarcinoma • LT is an emerging therapy for unresectable CC • 5-year survival rate from 33% to 45% Sotiropoulos GC, et al.Transplant Proc 2008 Heimbach JK, et al. Semin Liver Dis 2004 Rea DJ, etal. Ann Surg 2005 Mayo protocol 5 survival is 73% Charles B. Rosen, et al. Transplant International. 2010

  41. Metastatic and Other Tumors • Hepatocellular carcinoma and liver metastases can cause obstructive jaundice by direct extension into the perihilar bile ducts.Primary and secondary hepatic tumors can also produce biliary obstruction by metastasizing to hilar or pericholedochal lymph nodes.Hepatocellular carcinoma, colorectal carcinoma, and pancreatic carcinoma are the most common primary sites associated with biliary tract obstruction from lymph node metastases.

  42. THANKS

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