Extra hepatic cholangiocarcinoma: Symptoms, treatment and prognosis

Primary bile duct cancers are rare and generally aggressive entities. Historically, this disease was managed exclusively with surgical therapy with few long term survivors. However, with improvements in chemotherapeutics, targeted therapy, interventional radiology techniques and development of alternative surgical approaches, treatment has moved to utilization of a multidisciplinary care team employing multimodality treatments and has finally led to improvement in outcomes. These results must be met with measured optimism as these tumors are often elusive and diagnosis is frequently delayed which contributes to poor overall survival. Additionally, determining the appropriate treatment modality and sequence can also be challenging to physicians. Many may not be familiar with or have access to innovative techniques in diagnostics and treatments that are available. This chapter will discuss the clinical aspects of care of extrahepatic biliary cancers (excluding gallbladder cancer) with an emphasis on diagnosis and management. The clinical signs and symptoms suggestive of biliary cancer, the importance of the anatomic location of tumors and its effect on work-up, treatment and prognosis will be discussed. Advantages and disadvantages of different imaging techniques will be addressed. An evidence-based evaluation of newer therapeutic interventions and review of established procedural options will be explored. Examples include interventional radiology procedures, endoscopic procedures and various surgical interventions (including transplantation). Likewise, the role of chemotherapy, targeted therapies and radiation perioperatively will also be addressed. Lastly, for patients with metastatic/unresectable disease, options for treatment and/or palliation will be reviewed.