Review articleCurrent Surgical Management of Hilar and Intrahepatic Cholangiocarcinoma: The Role of Resection and Orthotopic Liver Transplantation
Section snippets
Epidemiology
CCA is a rare malignant disease, although the worldwide incidence of this disease is steadily increasing. In the United States, there was an estimated incidence of 3,000 cases of primary bile duct cancer in 2002. For unclear reasons, there has been a shift of proportion between the intrahepatic and extrahepatic forms of CCA during the past 2 decades. Although the incidence of extrahepatic CCA (EHC) is declining, there has been a significant increase in incidence and mortality rates of IHC
Classification
More than 90% of CCA are mucin-producing, well-differentiated adenocarcinoma. Based on growth characteristics, these tumors are identified as mass-forming, periductal-infiltration, or intraductal types. Anatomically, CCA can develop anywhere along the biliary tree and is classified as extrahepatic or intrahepatic tumors (Fig 1). The extrahepatic form is divided into 2 subtypes: the proximal (also known as perihilar, hilar, or Klatskin) tumor accounts for 60%–70% and the distal for 20%–30% of
Clinical Presentation
The typical age of presentation for CCA is the 7th decade of life, although those with predisposing risk factors such as primary sclerosing cholangitis (PSC), hepatolithiasis, liver flukes, or choledochal cyst can develop up to 2 decades earlier.9 CCA is usually silent until it presents with advanced disease. The clinical presentation is nonspecific, depending on the anatomic location of the predominant tumor. For IHC, common symptoms include abdominal pain, weight loss, malaise, and cachexia,
Preoperative Workup and Diagnosis
The preoperative workup for IHC and EHC primarily aims to accurately assess the tumor extent and feasibility to completely extirpate the tumor including all microscopically detectable disease (R0 resection). The diagnostic studies for IHC are in many aspects similar to that of other intrahepatic neoplasms such as HCC or hepatic metastases and should include a thorough search for a possible primary tumor to exclude metastatic nature of the liver lesion.
Serum tumor markers CA19-9, CEA, and CA-125
Management With Portal Vein Embolization and Biliary Drainage Before Resection
Once the resectability of the tumor has been determined, 2 major preoperative issues have to be addressed: the adequacy of functional reserve of the future liver remnant and those related bile duct obstruction. Resections of hilar CCA are often complex that require extended hemihepatectomies with excision of segment 1 to achieve R0 resection. In the majority of cases, the future liver remnant consists only of the left lateral segments (segments 2 and 3) with or without segment 4a, posing a
IHC
IHC or peripheral CCA is the second most common primary liver malignancy behind HCC. The etiology of IHC remains poorly understood, but several diseases that are associated with CCA are linked by a common pathologic condition, namely, chronic biliary inflammation. These include primary biliary cirrhosis, PSC, chronic hepatolithiasis, choledochal cyst, hepatitis C viral infection, and parasitic biliary infestation.
Complete resection is the only treatment that offers the possibility of long-term
Hilar CCA
Hilar CCA, also known as Klatskin tumor or proximal EHC, arises from the biliary confluence or the right or left hepatic ducts. Treatment for hilar CCA has remained challenging because of the lack of effective adjuvant treatment, the close proximity of the tumor to vital biliary and vascular structures as well as to other organs and a limited ability to achieve complete resection owing to the locally advanced nature of the tumor at presentation.65 The operative management of hilar CCA has
Summary
The management for CCA remains challenging because of the rarity and aggressive nature of the disease, lack of effective adjuvant therapy, as well as the diverse locations of tumor. In contrast with HCC, there has been no effective screening test for CCA such that patients oftentimes present with unresectable disease. Surgical extirpation of the tumor is the only chance for potential cure. During the last 2 decades, an R0 resection has constantly been reported the most important predictor of
References (104)
Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States
Hepatology
(2001)- et al.
Increase in primary liver cancer in the UK, 1979–94
Lancet
(1997) Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatisAn unusual tumor with distinctive clinical and pathological features
Am J Med
(1965)- et al.
Malignancies of the biliary tree
Curr Probl Surg
(1995) - et al.
The utility of CA 19-9 in the diagnoses of cholangiocarcinoma in patients without primary sclerosing cholangitis
Am J Gastroenterol
(2000) - et al.
Impact of integrated positron emission tomography and computed tomography on staging and management of gallbladder cancer and cholangiocarcinoma
J Hepatol
(2006) - et al.
18F-FDG-uptake of hepatocellular carcinoma on PET predicts microvascular tumor invasion in liver transplant patients
Am J Transplant
(2009) - et al.
A prospective comparison of digital image analysis and routine cytology for the identification of malignancy in biliary tract strictures
Clin Gastroenterol Hepatol
(2004) - et al.
Preoperative portal vein embolization for extension of hepatectomy indications
Hepatology
(1996) - et al.
Right trisegment portal vein embolization for biliary tract carcinoma: technique and clinical utility
Surgery
(2000)