Elsevier

Transplantation Proceedings

Volume 41, Issue 10, December 2009, Pages 4023-4035
Transplantation Proceedings

Review article
Current Surgical Management of Hilar and Intrahepatic Cholangiocarcinoma: The Role of Resection and Orthotopic Liver Transplantation

https://doi.org/10.1016/j.transproceed.2009.11.001Get rights and content

Abstract

Cholangiocarcinoma (CCA) is a rare but devastating malignancy that presents late, is notoriously difficult to diagnose, and is associated with a high mortality. Surgical resection is the only chance for cure or long-term survival. The treatment of CCA has remained challenging because of the lack of effective adjuvant therapy, aggressive nature of the disease, and critical location of the tumor in close proximity to vital structures such as the hepatic artery and the portal vein. Moreover, the operative approach is dictated by the location of the tumor and the presence of underlying liver disease. During the past 4 decades, the operative management of CCA has evolved from a treatment modality that primarily aimed at palliation to curative intent with an aggressive surgical approach to R0 resection and total hepatectomy followed by 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

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