Multidisciplinary management and the future of treatment in cholangiocarcinoma

ABSTRACT

Introduction: Cholangiocarcinoma is an often lethal and relatively rare cancer that arises from the epithelial lining of the biliary tree. Disease can arise throughout the liver, at the confluence of the right and left main hepatic ducts, or as the common bile duct runs toward the ampulla of Vater. Cholangiocarcinoma has a distinct geographic variability with the greatest incidence in the Far East. Worldwide, the incidence of cholangiocarcinoma appears to be increasing.

Areas covered: In this review, a comprehensive search of the peer-reviewed literature is completed to provide an update regarding the diagnostic adjuncts, clinical management, and areas of active research for this disease. There are no reliable screening tools available for clinical detection. Surgery is the only chance for cure. Chemotherapy is often used in the adjuvant setting or in patients with unresectable disease, though outcomes are generally poor. Newer approaches that include hepatic artery-based therapies and image guided radiation therapy appear to provide limited survival benefit.

Expert opinion: Maximizing survival following a diagnosis of cholangiocarcinoma relies on a multidisciplinary team of experts in medical oncology, radiation oncology, surgical oncology (principally hepatopancreatobiliary surgeons), and interventional radiology.

KEYWORDS:

• Cholangiocarcinoma
• neoadjuvant therapy
• adjuvant therapy
• stereotactic body radiation therapy

Article highlights

• Cholangiocarcinoma is a rare cancer that arises from malignant degeneration of the biliary epithelium anywhere along the biliary tree from the liver to the distal common bile duct.

• Once thought of as a single disease entity, cholangiocarcinoma is now recognized as a diverse set of tumors with significant genetic differences and variable prognosis.

• Surgery remains the only chance for cure.

• The current chemotherapeutic standard, gemcitabine in combination with cisplatin, is often well tolerated but rarely provides durable disease response. In the adjuvant setting, gemcitabine combined with capecitabine is commonly prescribed.

• Trans-arterial, liver-directed therapies such as chemoembolization or radioembolization have been used with modest prolongation of survival. The role of stereotactic body radiation therapy with photons and protons is emerging.

• Current research efforts are most commonly focused on elucidating the genetic basis of the disease, attempting to find biomarker correlates of disease response to current therapies, and attempts to modify the current chemotherapeutic gold standard to incrementally prolong overall survival.

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Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.