In patients with PSC, CCA is the most dreaded complication. The survival of patients with PSC plus CCA is greatly diminished. Neither a specific serum tumor marker nor any imaging studies have been proved to be 100% accurate in the detection of CCA. However, more recently developed techniques such as DIA and FISH have shown to improve on the results in patients with negative routine cytology. On the basis of these data, we now routinely use these studies for the diagnosis of CCA in PSC (Table 1). Patients should be followed yearly with MRI studies. If there is a rapid change in a focal stricture and/or the rapid onset of jaundice, we advise direct cholangiography (usually by endoscopic retrograde pancreatography), and brushings and biopsies of the dominant stricture. Where available, advanced cytologic studies for aneuploidy should be performed.
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