Molecular genetics and targeted therapeutics in biliary tract carcinoma

Table 1 Epidemiology and clinical features of the primary malignancies of the biliary tract

Type of malignancy	Incidence	Risk factors	Typical presentation	Diagnosis
CC	1-2 per 100000 population[88]	Increasing age[89]		CT or MRI: Mass lesion with contrast uptake during arterial and venous phases[89]
		Hispanic or Asian ethnicity[89]
		PSC[89]
		Helminth infection[89]
		Choledochal cyst[89]
		Thorotrast[89]
		Metabolic syndrome[89,90]
		Hepatobiliary stones[89]
		Viral hepatitis[89,90]
Intrahepatic	10% of CC[89]		Constitutional symptoms (fevers, night sweats, unintended weight loss)[89]	Differentiate from hepatocellular carcinoma via timing of contrast uptake[89]
Extrahepatic	90% of CC[90]		Painless jaundice[89,90]	ERCP with brushing can obtain sample for cytology
				EUS with FNA of lymph nodes can assess for metastasis
GBC	1-2 per 100000 population[91]	Increasing age[92]	Painless jaundice[92]	EUS: Allows for FNA and is considered definitive for staging[92]
		Female gender[92]
		Hispanic, Asian, or Eastern European heritage[92]
		Gallstones[92]	Constitutional symptoms (fevers, night sweats, unintended weight loss)[92]	CT or MRCP: Determines resectability
		Salmonella[92]
		Helicobacter pylori[92]
		PSC[92]
		Heavy metal exposure[92]
		Metabolic syndrome[92]

CC: Cholangiocarcinoma; EUS: Endoscopic ultrasound; ERCP: Endoscopic retrograde cholangiopancreaticography; GBC: Gallbladder carcinoma; MRCP: Magnetic resonance cholangiopancreaticography; PSC: Primary sclerosing cholangitis.