PSC runs an unpredictable clinical course leading to liver cirrhosis and terminal liver disease in the majority of affected individuals. The ideal time to perform colectomy in colitis ulcerosa patients is still unclear; so far, it is a matter of local preference and patient condition. The problem of identifying PSC patients with a particular risk for developing CCC is multifactorial. Diagnostic features are still lacking. Living related donor liver transplantation for PSC could reduce waiting time and thus secondary complications as well as drop-out probability due to malignancy [16]. Major surgery with simultaneous pancreatic head resection may be necessary in cases with extrahepatic duct infiltration in order to achieve no residual tumour (R0) status [15].

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