Ustundag Y, Bayraktar Y (2008) Cholangiocarcinoma: a compact review of the literature. World J Gastroenterol, 14(42):6458-6466
CODE: ICCYU08
Abstract: These tumors progress insidiously, and liver failure, biliary sepsis, malnutrition and cancer cachexia are general modes of death associated with this disease. CC is resectable for cure in only a small percentage of patients.
Epidemiology
CC is the second most common primary liver cancer after hepatocellular carcinoma (HCC) in most parts of the world. However, in some populations where HCC is uncommon, such as in Danish women, the prevalence of CC surpasses that of HCC[2].
Role of Clinics and Laboratory Tests in Diagnostic Evaluation
Intra-hepatic CCs present mostly with non-specific symptoms, such as abdominal pain, weight loss, malaise and decreased appetite. Occasionally, an incidental abdominal mass detected during physical examination or radiologic evaluation is the single finding. Mildly elevated alkaline phosphatase and normal bilirubin levels are noticed on laboratory testing. CA 19-9 can also be found increased. These tumors are generally confused with metastatic adenocarcinomas. Indeed, a liver mass with adenocarcinoma histology, without an obvious primary source, should be seriously considered as intra-hepatic CC. A needle biopsy of the dominant liver mass is a straightforward diagnostic approach in these patients. Exclusion of another primary source can usually be accomplished by systemic physical examinations, chest X-ray, and tomography of the abdomen and pelvis.
Contrast enhanced helical computerized tomography (CT) is very sensitive for detecting intra-hepatic CC larger than 1 cm. CT can also locate the site of obstruction and the presence of lymphadenopathy[33,34]. CT angiography can also detect vascular encasement[35]. Helical CT is only 60% correct in determining resectability.
Magnetic resonance imaging (MRI) with MR Cholangiopancreatography (MRCP) has mostly replaced CT in diagnosis and staging evaluation of CC[37-39]. MR investigations can detect the site and extent of tumor involvement in the absence of PSC. MR angiography can show vascular involvement in these cases[40]. Thus, MRI studies have the advantage of showing vascular anatomy, crosssectional imaging of the liver and cholangiography with a single technique and may exemplify an optimal imaging technique for this disease. However, in one study, MR cholangiography was reported to under-stage malignant hilar strictures in as high as 20% of patients[41].
Brush cytology has been reported to carry a very low diagnostic yield, ranging from 9%-24%. This was reported to be independent of the quantity of the specimen cellularity[46]. New technologies, such as the use of fluorescence in situ hybridization and digital image analysis methods, were reported to be more sensitive than the routine cytology. In one study, these technologies doubled the diagnostic accuracy of brush cytology[47].
Staging and Treatment Modalities
Resection and/or liver transplantation are the only curative options for CC. Accurate preoperative staging will determine the treatment approach in these patients.
Surgery is the most suitable option for patients with intra-hepatic CC. With curative surgery, three-year survival rates have been reported to be approximately 40%-60%[54]. An analysis of recent surgical series indicated that five-year survival data is around 40% (Table 3)[62-66]
Conclusion
CC is a devastating tumor with a high mortality rate. Its incidence is increasing and there is no new proven medical treatment modality. It is notorious as being difficult to diagnose as well as treat. Strategies are needed to detect these tumors at an early stage to apply radical curative therapy modalities. EUS-guided FNA is the most promising approach in this respect.
CODE: ICCYU08
Abstract: These tumors progress insidiously, and liver failure, biliary sepsis, malnutrition and cancer cachexia are general modes of death associated with this disease. CC is resectable for cure in only a small percentage of patients.
Epidemiology
CC is the second most common primary liver cancer after hepatocellular carcinoma (HCC) in most parts of the world. However, in some populations where HCC is uncommon, such as in Danish women, the prevalence of CC surpasses that of HCC[2].
Role of Clinics and Laboratory Tests in Diagnostic Evaluation
Intra-hepatic CCs present mostly with non-specific symptoms, such as abdominal pain, weight loss, malaise and decreased appetite. Occasionally, an incidental abdominal mass detected during physical examination or radiologic evaluation is the single finding. Mildly elevated alkaline phosphatase and normal bilirubin levels are noticed on laboratory testing. CA 19-9 can also be found increased. These tumors are generally confused with metastatic adenocarcinomas. Indeed, a liver mass with adenocarcinoma histology, without an obvious primary source, should be seriously considered as intra-hepatic CC. A needle biopsy of the dominant liver mass is a straightforward diagnostic approach in these patients. Exclusion of another primary source can usually be accomplished by systemic physical examinations, chest X-ray, and tomography of the abdomen and pelvis.
Contrast enhanced helical computerized tomography (CT) is very sensitive for detecting intra-hepatic CC larger than 1 cm. CT can also locate the site of obstruction and the presence of lymphadenopathy[33,34]. CT angiography can also detect vascular encasement[35]. Helical CT is only 60% correct in determining resectability.
Magnetic resonance imaging (MRI) with MR Cholangiopancreatography (MRCP) has mostly replaced CT in diagnosis and staging evaluation of CC[37-39]. MR investigations can detect the site and extent of tumor involvement in the absence of PSC. MR angiography can show vascular involvement in these cases[40]. Thus, MRI studies have the advantage of showing vascular anatomy, crosssectional imaging of the liver and cholangiography with a single technique and may exemplify an optimal imaging technique for this disease. However, in one study, MR cholangiography was reported to under-stage malignant hilar strictures in as high as 20% of patients[41].
Brush cytology has been reported to carry a very low diagnostic yield, ranging from 9%-24%. This was reported to be independent of the quantity of the specimen cellularity[46]. New technologies, such as the use of fluorescence in situ hybridization and digital image analysis methods, were reported to be more sensitive than the routine cytology. In one study, these technologies doubled the diagnostic accuracy of brush cytology[47].
Staging and Treatment Modalities
Resection and/or liver transplantation are the only curative options for CC. Accurate preoperative staging will determine the treatment approach in these patients.
Surgery is the most suitable option for patients with intra-hepatic CC. With curative surgery, three-year survival rates have been reported to be approximately 40%-60%[54]. An analysis of recent surgical series indicated that five-year survival data is around 40% (Table 3)[62-66]
Conclusion
CC is a devastating tumor with a high mortality rate. Its incidence is increasing and there is no new proven medical treatment modality. It is notorious as being difficult to diagnose as well as treat. Strategies are needed to detect these tumors at an early stage to apply radical curative therapy modalities. EUS-guided FNA is the most promising approach in this respect.
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