T1

The neoplasm within the ductal wall is defined as early bile duct cancer it represents about 10 of all cholangiocarcinoma and shows distinctive characteristics. In 52 of cases the neoplasms show a papillary aspect and in more than 60 are well differentiated. Lymph-node involvement is present in 0-2 of the cases and perineural invasion is present in 0-10 19,20 . The prognosis is good with a 5-year survival rate of 78 . According to gross type the survival rate of papillary and infiltrative...

S8 S1

The left trisectionectomy with caudate lobectomy is indicated for hilar cholan-giocarcinoma that involves the left intrahepatic bile ducts in continuity with the duct to the right anterior sector (S5 and S8). After concluding dissection of hepatoduodenal ligament as previously described, the left and middle hepatic arteries are tied and divided at their origins. The right artery is isolated very gently up to its second order bifurcation for anterior and posterior sectors. The branch for...

Intraoperative Assessment of Resectability

At the time of diagnosis, patients with intrahepatic cholangiocarcinoma (ICC) are frequently found to have disease beyond the limits of surgical therapy, such the presence of intrahepatic satellite nodules, vascular invasion, or regional lymph-nodes metastases. In such patients, the resectability rate varies from 19 to 74 1-4 . Moreover, exploratory laparotomy or R1 resection has a poor prognosis, with median patient survival of 5 months and postoperative complications of 17 4 . Consequently,...

Tumours

With reference to the bile ducts intrahepatic or hilar (Fig. 4) - Extent of Biliary Involvement. For hilar cholangiocarcinoma involvement of right hepatic duct - left hepatic bile duct - junction of right and left hepatic ducts - common bile duct (Bismuth classification). If more than one anatomical portion is involved, all involved portions should be recorded in the order of involvement, first indicating the portion in which the bulk of the tumour is located. Fig. 4a,b Hilar...

Ultrasound

Transabdominal ultrasound does not contribute any information to the evaluation of this parameter. However echoendoscopy appears to be useful in staging cholangiocarcinoma the lesion is detected as a hypoechoic mass within the lumen of the duct, frequently with infiltration of surrounding tissue. Sometimes the lesion is encased by hyperechoic tissue that is the expression of peritumoral fibrosis 4 . Tio et al. 5 reported a correct evaluation of T-stage in 85 of 43 cases with carcinoma of hilar...

Ultrasound Endoscopic Intraductal Transabdominal

Ultrasound represents the technique of choice for confirming obstructive jaundice. In fact it allows easy detection of the dilatation of the intra- and extrahep-atic biliary systems. However, although it shows high diagnostic reliability (80-94 ) in detecting biliary dilatation and the level of obstruction, its reliability in diagnosing the cause of obstruction decreases. Hilar cholangiocarcinoma may be presumed at ultrasound in presence of a hilar hypoechoic mass that has spread along the...

Magnetic Resonance Imaging

The MRI appearance of cholangiocarcinoma is that of a non-capsulated tumour, hypointense on T1-weighted images and hyperintense on T2-weighted images. The signal intensity of the tumour varies according to the amount of fibrosis, necrosis, and mucinous material within it. Central hypointensity may be seen on Fig. 4 Axial CT image during the arterial phase shows a hypodense-cen-tred mass on the left hepatic duct infiltrating the left hepatic artery Fig. 4 Axial CT image during the arterial phase...

Palliative Therapy Chemotherapy

Khan 9 , in the consensus document of guidelines for the diagnosis and treatment of cholangiocarcinoma, reported to date, a review of over 65 disparate studies using chemotherapy and or radiations suggests that was no strong evidence of survival benefit. However, most studies were small, lacked control groups (phase II) and were difficult to interpret. Data from a phase II study suggested that gemcitabine monotherapy is an active and well-tolerated treatment. Combinations of gemcitabine with...

Chemoradiation Therapy

Considering the radiosensitivity of some drugs such as 5-FU and gemcitabine, combining both therapies would appear hypothetically more efficient than sin gle treatment. However the data in the literature are divergent even for this therapeutic combination. In a group of 84 patients with cholangiocarcinoma (30 stages I and II, 54 stage III) who submitted to surgery alone and surgery associated with chemoradiation therapy (40 Gy + bolus 5-FU) Kim et al. 11 showed a 5-year survival of 36 in the 47...

Anatomy of the Portal Vein Branches

Usually few variants regard portal vein branches due to their early embryonic development during the gestation period. Three principal portal vein branching patterns in the hilar area are described by different authors - The common type (74-84 ) in which the anterior segmental branch joins the posterior to form the right branch of the portal vein. - The three branch type (8-12 ) in which the anterior segmental branch joins the portal vein confluence. - The left branch type (9-17 ) in which the...

TNM Staging System According to Uiccajcc 6th Edition

The sixth edition of TNM classification by Internal Union Against Cancer (UICC) and the sixth edition of the Manual for Staging of Cancer edited by American Joint Committee on Cancer were published in 2002 and used the same TNM classification and subdivision in stages 4 . Local extent of disease (T), nodal involvement (N) and presence of distant organ involvement (M) are determined by preoperative, intraoperative and pathological findings. T category evaluates local extent based on the degree...

Preoperative Assessment of Liver Function

Many elements contribute to the optimal selection of patients who will undergo surgery for hilar cholangiocarcinoma, including - Improvement of the preoperative diagnosis in the definition of size, site and tumour extent related to intrahepatic vascularisation - Findings of the intraoperative workup - Knowledge of the biological behavior of the tumour - Effects of biliary obstruction and jaundice - Effects of parenchymal inflammatory process and of cholangitis on clinical course and prognosis...

Surgical Anatomy of the Hepatic Hilus

The development of curative surgery for hilar cholangiocarcinoma is based on the precise knowledge of anatomy of the hepatic hilus and of the frequent anatomical variations that may be encountered. For these reasons it would be useful to point out some anatomical details, with particular regard to the anatomy of the hilar and caudate lobe area that are the crucial point of this surgery. Biliary ducts, arterial and portal vessels that are covered by connective tissue arising from the fusion of...

Drainage Cons

A critical evaluation of the indiscriminate use of preoperative biliary drainage has revealed functional limits and undeniable side effects - After biliary drainage bilirubinemia normalizes in only two-thirds of the treated cases 5,31 and 4-8 weeks are required for complete disappearance of jaundice 32 . Watanapa 33 has shown in preoperative and postoperative functional studies that the liver resumes its normal functions 6 weeks after surgical operation entailing jaundice drainage with...

Prognostic Significance of Tnm Uiccajcc Classification

Until now, few reports have valued the prognostic significance of TNM classification for hilar cholangiocarcinoma. The new edition of TNM staging (6th edition of UICC AJCC) focuses on two aspects vascular invasion and the presence of lymph-node metastases 45 . The prognostic significance of staging grouping according to UICC AJCC is still debated in a recent study, Zervos did not show any correlation between stage and survival in 42 patients submitted to surgical resection 12 nor did Hemming's...

Anatomy of the Bile Duct Branches

At the hepatic hilum the right and left hepatic ducts meet in the biliary confluence. Anatomical variants of the confluence are very common so that a regular anatomic confluence is present in no more than the two-thirds of cases. The left lateral segmental ducts (B2 and B3) generally join at the level of the umbilical fissure situated posterior to the umbilical portion. Rarely (6 of cases) the duct for segment 3 (B3) runs caudally to the umbilical portion of the left portal vein with a direct...

N Category

The presence of lymph-node metastases in ICC is a major negative prognostic factor. The prevalence of nodal metastases is high and varies from 7 to 73 2,17,18,27,28 . Long-term prognosis in patients with lymph-node metastases is poor (5-year survival of 0-17 Table 3). Table 3 Survival according to lymph-node status Table 3 Survival according to lymph-node status Japanese nationwide follow-up survey bSurvival for patients with < 3 positive lymph nodes was 50 , whereas it was 0 for > 3...

Comparison between 5th and 6th Edition of Tnm Uiccajcc

The last two staging editions of TNM, the 5th and 6th editions, present different criteria for classification of T and N categories. In the T category of the 5th edition all neoplasms involving adjacent structures were classified as T3 instead in the last edition T3 has been divided based on the structures involved T3 for lesions that invade per contiguity the liver, gallbladder, pancreas, or the vessels unilaterally and T4 for the lesions invading other adjacent organs (colon, stomach and...

Hepatectomy with Portal Resection and Reconstruction

Vein Resection Case Burn

The infiltration of portal bifurcation is not so rare in hilar cholangiocarcinoma, due to the close relationship of the biliary confluence with the portal system. In these cases the use of hepatectomy with en-bloc portal vein resection has been advocated by several authors, to increase the curative resections. When curative resection was achieved using portal resection and reconstruction, patients survived significantly longer than unresected patients 2 . Fig. 12 Central hepatic...

Gross Type

The classification proposed by the Liver Cancer Study Group of Japan (LCSGJ) distinguishes three different macroscopic types mass forming (MF), periductal-infiltrating (PI), and intraductal growth (IG) 1 . These three macroscopic forms reflect different biological behaviours and neoplastic diffusion. MF cholangio-carcinoma is associated with early portal invasion and intra hepatic metastases the PI type is characterised by invasion of the biliary tree and of Glisson's sheath, with preferential...

Left Medial Sectionectomy with Caudate Lobectomy S4 S1

The operation begins with hilar time, complete mobilisation of the liver and caudate lobe as described in the isolated caudate lobectomy. The parenchymal dissection is performed with anatomic resection of caudate lobe and segment 4. Transection of the left biliary duct is immediately on the right of the falciform ligament on segment S2 and S3 while on the right side falls just on the two biliary ducts the anterior and the posterior. The bilioenteric continuity is restored with multiple...

Anatomy of the Hepatic Artery Branches

The left hepatic artery enters the liver on the left side of Rex's recessus the middle hepatic artery, through the right side of Rex's recessus the right hepatic artery more frequently runs between the portal vein and the bile duct, posterior to the confluence. The right hepatic artery divides into the anterior branch that runs between the bile duct and the portal vein, and posterior branch that turns caudally to the right portal vein and enters the liver (see Fig. 1) 3 . Variations of the...

Independent Caudate Lobectomy S1

The isolated resection of the caudate lobe associated with resection of the extra-hepatic biliary tract represents a fairly rare indication and is limited to cases with neoplasm confined clearly inside the confluence between right and left ducts with involvement of caudate branches. The resection of the caudate lobe due to its position always requires the complete mobilization of the liver. The operation begins with hepatoduodenal ligament dissection, lymph node clearing and transection of the...

Right Anterior Sectionectomy with Caudate Lobectomy S5S8 S1

This operation also begins with the hilar phase, complete mobilisation of the liver and caudate lobe as described before. The arterial and portal branches to the caudate lobe that arise from the main right and left branches are ligated. After cholecystectomy and transection of the distal bile duct on free margin, the biliary tract is reflected upward and the right vascular elements are isolated up to the bifurcation in anterior and posterior branches. The arterial braches for the right anterior...

Surgical Anatomy of the Caudate Lobe

Caudate Lobe Liver Diagrams

Knowledge of anatomy of the caudate lobe and its relationship with the porta hepatis area are mandatory for a correct surgical approach to hilar cholangiocar-cinoma. In fact, the surgical treatment of this disease requires en-bloc resection of the caudate lobe. Currently the caudate lobe is divided into three parts, according to Couinaud's definition 6 - Segment 1 (S1), or caudate lobe, in the strict sense of the word, which corresponds to the portion with left development - Segment 9 (S9),...

Right Hepatectomy with Caudate Lobectomy S4a S5 S6 S7 S8 S1

Right hepatectomy with caudate lobectomy is indicated for tumours involving the right anterior and posterior sectorial bile ducts with sparing of the left medial segmental bile duct to segment 4. The resection entails the right hepatic lobe, caudate lobe and, if indicated, the caudal portion of segment 4 (4a) to obtain an adequate margin of biliary ducts. After mobilisation of duodenum and retropancreatic lymph node dissection, the distal bile duct is transected in its suprapancreatic portion...

Central Hepatic Resections

Central hepatectomies consisting of left medial sectionectomy with caudate lobectomy (S1+S4), right anterior sectionectomy with caudate lobectomy (S1+S5, S8) and central hepatic bisegmentectomy with caudate lobectomy (S1+S4, S5, S8), are proposed mainly by Japanese authors 5,31,32 . These parenchyma-preserving hepatectomies find their premise in limited hepatic resection to minimise postoperative risk of hepatic failure in high-risk patients, still maintaining a potentially curative R0...

Right Trisectionectomy with Caudate Lobectomy S4 S5 S6 S7 S8 S1

The right trisectionectomy with caudate lobectomy is indicated for hilar cholan-giocarcinoma that involves the right intrahepatic bile duct in continuity with the left medial segmental duct (segment 4). The phases of retropancreatic and hepatoduodenal ligament lymph node dissection, of distal bile duct section and mobilization of caudate lobe are similar to the abovementioned for right hepatectomy. Right and middle hepatic artery and right portal vein are ligated and divided. Mobilisation of...

Left Hepatectomy with Caudate Lobectomy S2 S3 S4 S1

The extended left hepatectomy with caudate lobectomy finds its indication in neoplasms of the biliary confluence that extend to the left intrahepatic bile duct. After hilar preparation and ligation of arterial and portal branches for caudate lobe, the left and middle hepatic artery and left portal vein are tied. The left lobe is completely mobilised with section of left triangular and coronary ligaments. The lesser omentum is incised and the left part of the caudate lobe is mobilised from down...

Choice of Treatment

The palliation strategy depends on when the diagnosis of unresectability is made in fact, when it is obtained preoperatively or at preliminary laparoscopy there are two options the endoscopic and the percutaneous route. Instead, when the condition of unresectability is verified intraoperatively there are different options surgical management, maintenance of the biliary drainage, if already positioned, intraoperative trans-tumoral catheterization or postoperative positioning of endoscopic or...

Macroscopic Growth Pattern

On macroscopic assessment neoplasms would be classified on the basis of the type of growth of the tumour inside the biliary duct - Papillary type included pedunculated and sessile tumours thicker than 2 mm Expanding pattern Infiltrating pattern Expanding pattern Infiltrating pattern Infiltrating pattern flat type with infiltrating pattern entails diffuse infiltrating type Evaluation of the extent of the disease involves five different parameters serosa, hepatic parenchyma, pancreas, portal vein...

Recurrence

Recurrence after R0 resection is frequent, ranging from 38 to 82 it is usually early and most often occurs within 2 years postoperatively. Huang reported a median time of disease relapse of 13 months 8 . The most frequent sites of recurrence are intrahepatic 74 , peritoneal 22 , bone 11 , lymph node 11 and, less frequently, distantly lung, abdominal wall 34 . The factors related to the onset of recurrence are many and are associated with gross tumour type and extent of disease. The former...

Indications for Lymphadenectomy

The rational extent of radical lymphadenectomy for ICC has not been clearly defined and there is no consensus on the role of lymph-node dissection 18,26 . The incidence of lymph-node metastasis in ICC is 43-62 1,2,7,10,15,21 . Several series have shown that one of the strongest prognostic factors in ICC is lymph-node involvement 17,25,27,28 . All of these studies reported that no patients with lymph-node involvement survived for more than 3 years after surgery. Inoue 17 stated that lymph-node...

Gazzaniga Staging System

Hilar Cholangiocarcinoma

The classification proposed by Gazzaniga in 1985 was the first to consider biliary and vascular invasion 11 . Based on preoperative workup two elements are evaluated extent of biliary involvement according to Bismuth-Corlette and portal and arterial vascular infiltration. Fig. 5 Staging system according to JSBS Japanese Society of Biliary Surgery 10 H, Hepatic metastases H0 absent H1 limited to a lobe H2 few metastases to both lobes, H3 many metastases to both lobes P, peritoneal metastases P0...