Revised AJCC Classification of Extrahepatic Bile Duct Tumors Definitions Anatomy of the Bile Ducts right hepatic duct left hepatic duct hepatic hilum perihilar bile ducts proximal common duct (proximal hepatic duct) cystic duct middle common duct common bile duct distal bile duct distal common duct ampulla of Vater 7th AJCC classification Definitions AJCC Classification Extrahepatic Bile Duct Tumors 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 6th edition (2002) Separated into perihilar bile duct and distal bile duct tumors in the 7th edition (2010) 6th AJCC Classification Extrahepatic Bile Duct Tumor Primary tumor (T) Stage 0 Tis N0 M0 Stage IA T1 N0 M0 Stage IB T2 N0 M0 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 Stage IIA T3 N0 M0 T3 Tumor invades the liver, gallbladder, pancreas, and/or unilateral branches of the portal vein (right or left) or hepatic artery (right or left) Stage IIB T1-3 N1 M0 Stage III T4 Any N M0 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 Stage IV Any T Any N M1 7th AJCC Classification Definitions of TNM Perihilar Bile Duct Tumor 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 Distal Bile Duct Tumor 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 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 7th AJCC Classification Definitions of TNM Perihilar Bile Duct Tumor Regional lymph nodes (N) NX Regional lymph nodes cannot be assessed N0 No 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 Distant metastasis (M) Distal Bile Duct Tumor 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 M0 No distant metastasis M1 Distant metastasis M1 Distant metastasis 7th AJCC Classification Anatomic Stage/Prognostic Groups Perihilar Bile Duct Tumor Distal Bile Duct Tumor Stage 0 Tis N0 M0 Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage IA T1 N0 M0 Stage II T2a-b N0 M0 Stage IB T2 N0 M0 Stage IIIA T3 N0 M0 Stage IIA T3 N0 M0 Stage IIIB T1-3 N1 M0 Stage IIB T1-3 N1 M0 Stage IVA T4 N0-1 M0 Stage III T4 Any N M0 Stage IVB Any T N2 Any N M0 M1 Stage IV Any T Any N M1 Summary Perihilar Bile Duct Tumors T1: confined to the bile duct T2: beyond the wall of the bile duct wall T2b: invasion of adjacent hepatic parenchyma (T3 in the 6th edition) T3: unilateral vascular invasion T4: bilateral biliary and/or vascular invasion Regional lymph node metastasis: reclassified as stage IIIB (stage IIB in the 6th edition) T4: unresectable based on local invasion (IVA) or distant disease (IVB) Summary Distal Bile Duct Tumors 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 6th edition) Invasion of the portal vein and hepatic artery: excluded in classification (T3 or T4 in the 6th edition) No criteria on the longitudinal involvement Radiologic Staging Perihilar Bile Duct Tumors Stage I • CT: wall thickening from left hepatic duct to distal common duct • Surgery: left major hepatobiliary resection, pathology: T1N0M0 • Major surgery was performed due to extensive longitudinal involvement Radiologic Staging Perihilar Bile Duct Tumors Stage II • CT: wall thickening from both hepatic ducts to proximal common duct, periductal invasion (+) • Op: extended left hemihepatectomy, pathology: T2aN0M0 Radiologic Staging Perihilar Bile Duct Tumors Stage II • CT: wall thickening and mass invading to adjacent hepatic parenchyma, from left hepatic duct to hepatic hilum • Surgery: extended left hemihepatectomy, pathology: T2bN0M0 • T3 in the 6th edition, T2b in the 7th edition Radiologic Staging Perihilar Bile Duct Tumors Stage IIIA • CT: polypoid mass from both hepatic ducts to hepatic hilum, invasion to right hepatic artery • Surgery: segmental resection due to old age, pathology: T3N0M0 • Upstaged from stage IIA (T3N0M0) to stage IIIA (T3N0M0) in case of unilateral vascular invasion Radiologic Staging Perihilar Bile Duct Tumors Stage IIIB • CT: wall thickening from hepatic hilum to proximal common duct, lymph node (+) • Surgery: segment resection and lymph node dissection, pathology: T2aN1Mo • Upstaged from stage IIB (T2N1M0) to IIIB (T2aN1M0) due to regional lymph node metastasis Radiologic Staging Distal Bile Duct Tumors Stage IA • CT: polypoid mass from mid to distal, pericholedochal invasion (-) • Surgery: PPPD, pathology: T1N0M0 Stage IB • CT: wall thickening from mid to distal, pericholedochal invasion (+) • Surgery: PPPD, pathology: T2N0M0 Radiologic Staging Distal Bile Duct Tumors Stage IIA • CT: wall thickening involving distal common duct, pancreatic invasion (+) • Surgery: PPPD and lymph node dissection, pathology: T3N0M0 • Pancreatic invasion: T3 in 6th and 7th editions Radiologic Staging Distal Bile Duct Tumors Stage IIA • CT: wall thickening from distal common duct, pancreatic and duodenal invasion (+) • Surgery: PPPD and lymph node dissection, pathology: T3N0M0 • Downstaged from stage IV (T4N0M0) to stage IIA (T3N0M0) due to duodenal invasion Radiologic Staging Distal Bile Duct Tumors Stage IIB • CT: polypoid mass from mid to distal common duct, pancreatic invasion (+), regional lymph node (+) • Surgery: PPPD, pathology: T3N1M0 Radiologic Staging Distal Bile Duct Tumors Stage IIB • CT: wall thickening from proximal to distal common duct, duodenal and pancreatic invasion (+), regional lymph node (+) • Surgery: PPPD, pathology: T3N1M0 • Downstaged from stage IV (T4N1M0) to stage IIB (T3N1M0) due to duodenal invasion Radiologic Staging Distal Bile Duct Tumors Stage IIB • CT: wall thickening from middle to distal common duct, pancreatic invasion (+), regional lymph node (+) • Surgery: PPPD and portal vein resection, pathology: T3N1M0 • Portal vein invasion: T4 in the 6th edition, not defined T3 in the 7th edition (maybe T3 as other adjacent organ) Limitations Perihilar Bile Duct Tumors False Negative Image Interpretation • Invasion to hepatic parenchyma: CT (-), pathology (+) Limitations Perihilar Bile Duct Tumors False Positive Image Interpretation • Invasion to right hepatic artery: CT (+), pathology (-) Limitations Perihilar Bile Duct Tumors Indefinite Staging System • CT: Pancreatic invasion (+) • CT: Gallbladder invasion (+) • T stage of pancreatic and gallbladder invasion: not clearly defined Limitations Distal Bile Duct Tumors False Negative Image Interpretation • Pancreatic invasion: CT (-), pathology (+) Limitations Distal Bile Duct Tumors False Positive Image Interpretation • Invasion beyond bile duct wall: CT (+), pathology (-) Limitations Distal Bile Duct Tumors Indefinite Staging System • Main portal vein invasion: CT (-), pathology (+) • Main portal invasion: T4 in the 6th edition, not clearly defined in the 7th edition (maybe T3 as other adjacent organ) New Staging Perihilar Cholangiocarcinoma Proposed Classification System 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 < 1 cm, 1-3 cm, > 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 DeOliveira MI, et al. Hepatology 2011;53:1363-1371