1. Revised AJCC Classification of Extrahepatic Bile Duct Tumors

2. Anatomy of the Bile Ducts DefinitionsDefinitions hepatic hilum proximal common duct (proximal hepatic duct) cystic duct ampulla of Vater distal bile duct perihilar bile ducts 7 th AJCC classification middle common duct common bile duct distal common duct left hepatic duct right hepatic duct

3. AJCC Classification DefinitionsDefinitions  Including bile duct tumors arising from the hepatic hilum of the right and left hepatic ducts to the distal common duct above the ampulla of Vater in the 6 th edition (2002)  Separated into perihilar bile duct and distal bile duct tumors in the 7 th edition (2010)

4. Primary tumor (T) TX Primary tumor cannot be assessed T0 No evidence of primary tumor Tis Carcinoma in situ T1 Tumor confined to the bile duct histologically T2 Tumor invades beyond the wall of the bile duct T3 Tumor invades the liver, gallbladder, pancreas, and/or unilateral branches of the portal vein (right or left) or hepatic artery (right or left) T4 Tumor invades any of the following: main portal vein or its branches bilaterally, common hepatic artery, or other adjacent structures, such as the colon, stomach, duodenum, or abdominal wall Extrahepatic Bile Duct Tumor 6 th AJCC Classification Stage 0TisN0M0 Stage IAT1N0M0 Stage IBT2N0M0 Stage IIAT3N0M0 Stage IIBT1-3N1M0 Stage IIIT4Any NM0 Stage IVAny TAny NM1

5. Definitions of TNM 7 th AJCC Classification Primary tumor (T) TX Primary tumor cannot be assessed T0 No evidence of primary tumor Tis Carcinoma in situ T1 Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue T2a Tumor invades beyond the wall of the bile duct to surrounding adipose tissue T2b Tumor invades adjacent hepatic parenchyma T3 Tumor invades unilateral branches of the portal vein or hepatic artery T4 Tumor invades main portal vein or its branches bilaterally; or the common hepatic artery; or the second-order biliary radicals bilaterally; or unilateral second-order biliary radicals with contralateral portal vein or hepatic artery involvement Primary tumor (T) TXPrimary tumor cannot be assessed T0No evidence of primary tumor TisCarcinoma in situ T1 Tumor confined to the bile duct histologically T2 Tumor invades beyond the wall of the bile duct T3 Tumor invades the gallbladder, pancreas, duodenum, or other adjacent organs without involvement of the celiac axis, or the superior mesenteric artery T4 Tumor involves the celiac axis, or the superior mesenteric artery

6. Definitions of TNM 7 th AJCC Classification Regional lymph nodes (N) NX Regional lymph nodes cannot be assessed N0No regional lymph node metastasis N1 Regional lymph node metastasis (including nodes along the cystic duct, common bile duct, hepatic artery, and portal vein) N2 Metastasis to periaortic, pericaval, superior mesenteric artery, and/or celiac artery lymph nodes Regional lymph nodes (N) NX Regional lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Regional lymph node metastasis N2 Metastasis to periaortic, pericaval, superior mesenteric artery, and/or celiac artery lymph nodes Distant metastasis (M) M0 No distant metastasis M1 Distant metastasis Distant metastasis (M) M0 No distant metastasis M1 Distant metastasis

7. Anatomic Stage/Prognostic Groups 7 th AJCC Classification Stage 0TisN0M0 Stage IT1N0M0 Stage IIT2a-bN0M0 Stage IIIAT3N0M0 Stage IIIBT1-3N1M0 Stage IVAT4N0-1M0 Stage IVBAny T N2 Any N M0 M1 Stage 0TisN0M0 Stage IAT1N0M0 Stage IBT2N0M0 Stage IIAT3N0M0 Stage IIBT1-3N1M0 Stage IIIT4Any NM0 Stage IVAny TAny NM1

8. Perihilar Bile Duct Tumors SummarySummary  T1: confined to the bile duct T2: beyond the wall of the bile duct wall  T2b: invasion of adjacent hepatic parenchyma (T3 in the 6 th edition)  T3: unilateral vascular invasion  T4: bilateral biliary and/or vascular invasion  Regional lymph node metastasis: reclassified as stage IIIB (stage IIB in the 6 th edition)  T4: unresectable based on local invasion (IVA) or distant disease (IVB)

9. Distal Bile Duct Tumors SummarySummary  T1: confined to the bile duct T2: beyond the wall of the bile duct wall  T3: includes adjacent organs (invasion of adjacent organs such as the colon, stomach, duodenum or abdominal wall: classified as T4 in the 6 th edition)  Invasion of the portal vein and hepatic artery: excluded in classification (T3 or T4 in the 6 th edition)  No criteria on the longitudinal involvement

10. Perihilar Bile Duct Tumors Radiologic Staging CT: wall thickening from left hepatic duct to distal common duct CT: wall thickening from left hepatic duct to distal common duct Surgery: left major hepatobiliary resection, pathology: T1N0M0 Surgery: left major hepatobiliary resection, pathology: T1N0M0 Major surgery was performed due to extensive longitudinal involvement Major surgery was performed due to extensive longitudinal involvement

11. CT: wall thickening from both hepatic ducts to proximal common duct, periductal invasion (+) CT: wall thickening from both hepatic ducts to proximal common duct, periductal invasion (+) Op: extended left hemihepatectomy, pathology: T2aN0M0 Op: extended left hemihepatectomy, pathology: T2aN0M0 Perihilar Bile Duct Tumors Radiologic Staging

12. CT: wall thickening and mass invading to adjacent hepatic parenchyma, from left hepatic duct to hepatic hilum CT: wall thickening and mass invading to adjacent hepatic parenchyma, from left hepatic duct to hepatic hilum Surgery: extended left hemihepatectomy, pathology: T2bN0M0 Surgery: extended left hemihepatectomy, pathology: T2bN0M0 T3 in the 6 th edition, T2b in the 7th edition T3 in the 6 th edition, T2b in the 7th edition Perihilar Bile Duct Tumors Radiologic Staging

13. CT: polypoid mass from both hepatic ducts to hepatic hilum, invasion to right hepatic artery CT: polypoid mass from both hepatic ducts to hepatic hilum, invasion to right hepatic artery Surgery: segmental resection due to old age, pathology: T3N0M0 Surgery: segmental resection due to old age, pathology: T3N0M0 Upstaged from stage IIA (T3N0M0) to stage IIIA (T3N0M0) in case of unilateral vascular invasion Upstaged from stage IIA (T3N0M0) to stage IIIA (T3N0M0) in case of unilateral vascular invasion Perihilar Bile Duct Tumors Radiologic Staging

14. CT: wall thickening from hepatic hilum to proximal common duct, lymph node (+) CT: wall thickening from hepatic hilum to proximal common duct, lymph node (+) Surgery: segment resection and lymph node dissection, pathology: T2aN1Mo Surgery: segment resection and lymph node dissection, pathology: T2aN1Mo Upstaged from stage IIB (T2N1M0) to IIIB (T2aN1M0) due to regional lymph node metastasis Upstaged from stage IIB (T2N1M0) to IIIB (T2aN1M0) due to regional lymph node metastasis Perihilar Bile Duct Tumors Radiologic Staging

15. Distal Bile Duct Tumors CT: polypoid mass from mid to distal, pericholedochal invasion (-) CT: polypoid mass from mid to distal, pericholedochal invasion (-) Surgery: PPPD, pathology: T1N0M0 Surgery: PPPD, pathology: T1N0M0 CT: wall thickening from mid to distal, pericholedochal invasion (+) CT: wall thickening from mid to distal, pericholedochal invasion (+) Surgery: PPPD, pathology: T2N0M0 Surgery: PPPD, pathology: T2N0M0 Radiologic Staging

16. Distal Bile Duct Tumors CT: wall thickening involving distal common duct, pancreatic invasion (+) CT: wall thickening involving distal common duct, pancreatic invasion (+) Surgery: PPPD and lymph node dissection, pathology: T3N0M0 Surgery: PPPD and lymph node dissection, pathology: T3N0M0 Pancreatic invasion: T3 in 6 th and 7 th editions Pancreatic invasion: T3 in 6 th and 7 th editions Radiologic Staging

17. Distal Bile Duct Tumors CT: wall thickening from distal common duct, pancreatic and duodenal invasion (+) CT: wall thickening from distal common duct, pancreatic and duodenal invasion (+) Surgery: PPPD and lymph node dissection, pathology: T3N0M0 Surgery: PPPD and lymph node dissection, pathology: T3N0M0 Downstaged from stage IV (T4N0M0) to stage IIA (T3N0M0) due to duodenal invasion Downstaged from stage IV (T4N0M0) to stage IIA (T3N0M0) due to duodenal invasion Radiologic Staging

18. Distal Bile Duct Tumors CT: polypoid mass from mid to distal common duct, pancreatic invasion (+), regional lymph node (+) CT: polypoid mass from mid to distal common duct, pancreatic invasion (+), regional lymph node (+) Surgery: PPPD, pathology: T3N1M0 Surgery: PPPD, pathology: T3N1M0 Radiologic Staging

19. Distal Bile Duct Tumors CT: wall thickening from proximal to distal common duct, duodenal and pancreatic invasion (+), regional lymph node (+) CT: wall thickening from proximal to distal common duct, duodenal and pancreatic invasion (+), regional lymph node (+) Surgery: PPPD, pathology: T3N1M0 Surgery: PPPD, pathology: T3N1M0 Downstaged from stage IV (T4N1M0) to stage IIB (T3N1M0) due to duodenal invasion Downstaged from stage IV (T4N1M0) to stage IIB (T3N1M0) due to duodenal invasion Radiologic Staging

20. Distal Bile Duct Tumors CT: wall thickening from middle to distal common duct, pancreatic invasion (+), regional lymph node (+) CT: wall thickening from middle to distal common duct, pancreatic invasion (+), regional lymph node (+) Surgery: PPPD and portal vein resection, pathology: T3N1M0 Surgery: PPPD and portal vein resection, pathology: T3N1M0 Portal vein invasion: T4 in the 6 th edition, not defined T3 in the 7 th edition (maybe T3 as other adjacent organ) Portal vein invasion: T4 in the 6 th edition, not defined T3 in the 7 th edition (maybe T3 as other adjacent organ) Radiologic Staging

21. LimitationsLimitations Perihilar Bile Duct Tumors Invasion to hepatic parenchyma: CT (-), pathology (+) Invasion to hepatic parenchyma: CT (-), pathology (+)

22. LimitationsLimitations Perihilar Bile Duct Tumors Invasion to right hepatic artery: CT (+), pathology (-) Invasion to right hepatic artery: CT (+), pathology (-)

23. LimitationsLimitations Perihilar Bile Duct Tumors CT: Pancreatic invasion (+) CT: Pancreatic invasion (+) CT: Gallbladder invasion (+) CT: Gallbladder invasion (+) T stage of pancreatic and gallbladder invasion: not clearly defined T stage of pancreatic and gallbladder invasion: not clearly defined

24. LimitationsLimitations Distal Bile Duct Tumors Pancreatic invasion: CT (-), pathology (+) Pancreatic invasion: CT (-), pathology (+)

25. LimitationsLimitations Distal Bile Duct Tumors Invasion beyond bile duct wall: CT (+), pathology (-) Invasion beyond bile duct wall: CT (+), pathology (-)

26. LimitationsLimitations Distal Bile Duct Tumors Main portal vein invasion: CT (-), pathology (+) Main portal vein invasion: CT (-), pathology (+) Main portal invasion: T4 in the 6 th edition, not clearly defined in the 7 th edition (maybe T3 as other adjacent organ) Main portal invasion: T4 in the 6 th edition, not clearly defined in the 7 th edition (maybe T3 as other adjacent organ)

27. New Staging Perihilar Cholangiocarcinoma DeOliveira MI, et al. Hepatology 2011;53:1363-1371 Bile duct Common bile duct, hepatic duct confluence, right hepatic duct, left hepatic duct, right and left hepatic duct Remnant liver volume No information, percentage of total volume after resection Tumor size 3 cm Underlying liver disease Fibrosis, NASH, PSC Tumor form Sclerosing, mass-forming, sclerosing and mass-forming, polypoid Lymph nodes No LN involvement, hilar and HA LN involvement, periaortic LN involvement Involvement of portal vein No portal involvement, main PV, PV bifurcation, right PV, left PV, right and left PVs Metastasis No distant metastasis, distant metastasis Involvement of hepatic artery No arterial involvement, proper HA, HA bifurcation, right HA, left HA, right and left HA