Recent Trends inDiagnosis and Treatment of Cholangiocarcinoma

Abstract

Cholangiocarcinoma (CCA) is the term applied to the primary malignant neoplasms arising from the biliary tract (it was first described by Durand - Fardel in 1840), it is the second most common primary hepatic malignancy, after hepatocellular carcinoma. According to the localization, CCA's are classified into intrahepatic (iCCA), perihilar (pCCA) and distal (dCCA), in more than 90% of cases, this tumor is histologically classified as an adenocarcinoma. Cholangiocarcinoma is difficult to diagnose, and the mortality rate is high, whereas, a modest delay in the diagnosis and initialization of treatment, will be reflected instantly in the prognosis of a given case, cholangiocarcinoma is difficult to be diagnosed in part because of its relative rarity, and because it is clinically silent until it becomes an advanced disease with obstructive symptoms. Over the past few decades, remarkable advances in imaging technology have been made that allow more accurate diagnosis of biliary tract diseases and better planning of surgical procedures and other interventions aimed at managing these conditions. Diagnosis of CCA should not rely solely on serum tumour marker measurements; CA 19-9 remains the most widely used serum tumour marker for suspected CCA, but does not exhibit high accuracy. It should be measured after relief of biliary obstruction. Brush cytology specimens via cholangioscopy are of high beneficial impact on the accuracy of diagnosis, Flourescence In-Situ Hybridization (FISH) may enhance the diagnostic sensitivity of cytology samples. To rule out metastatic disease, contrast CT of the abdomen, chest and pelvis should be carried out on all patients, particularly if resection is being considered. The treatment for CCA remains a challenge because of the aggressive nature of the disease and the absence of effective treatments besides surgical resection (HR) and liver transplantation (LTx). In iCCA, HR remains the treatment of choice whereas with concomitant liver disease such as cirrhosis or PSC, LTx is the only option. Surgical resection of tumors with negative margins (R0) is the best option for all subtypes of CCA, hence, surgery is the only curative treatment for cholangiocarcinoma, and so unless we can diagnose cholangiocarcinoma early and recommend complete surgical resection, there is no curative therapy. However, there are several restrictions on which people are eligible for surgery; operative techniques have been improved as a result of a better understanding of biliary and hepatic anatomy and physiology. Moreover, the continuing evolution of minimally invasive surgery has promoted the gradual adoption of laparoscopic approaches to these complex operations. Assessment of resectability, as well as venous and arterial invasion, is best accomplished using radiographic studies such as CT and/or MRI. Occasionally, removal of a pCCA or dCCA requires a pancreaticoduodenectomy (Whipple’s procedure). In unresectable tumors, surgical bypass is performed and biliary stents are placed. One important cause of death in this disease is biliary sepsis secondary to obstruction of the bile duct and uncontrolled Cholangitis. The continuing evolution of minimally invasive surgery has promoted the gradual adoption of laparoscopic approaches to these complex operations. Summarizing algorithms of recent protocols that are currently adopted in managing cholangiocarcinoma, included in the last section of this review (annex 2), offer a highly organized and hopefully effective plans recommended for proper management of this disease.