At present, PSC is the fifth most important indication for liver transplantation in the United States . There are two major difficulties associated with predicting the course of the disease in each individual case as well as the increased risk for hepatobiliary malignancies, primarily cholangiocellular carcinoma (CCC). The risk for CCC is highest during the first year of diagnosis and is not linked to the stage of disease [6, 7]. A PSC patient with cirrhosis and a liver tumour should also be evaluated for possible hepatocellular carcinoma (HCC) and in this case considered for Ltx if no extrahepatic metastases are detected and the tumour is within accepted criteria. Liver resection for HCC is rarely indicated in a PSC patient with advanced liver disease, as previous hepatobiliary surgery is a negative prognostic factor for transplant candidates .
The problem of identifying PSC patients with a high risk for developing CCC has not been resolved so far. No specific tumour markers exist. This has a great impact because CCC has traditionally been considered a contraindication to Ltx. Experience with CCC in patients undergoing Ltx is limited, and treatment guidelines are still lacking. The value of routine brush cytology obtained during endoscopic retrograde cholangiography remains unclear. Patients with severe dysplasia in cytologic smears should be considered as running a very high risk for CCC development and therefore evaluated to exclude possible systemic malignant disease. No consensus exists as to what imaging modality should be preferred in the management of these patients. Positron emission tomography (PET) is promising but its exact role remains unclear .
Studies indicate that Ltx results in highly selected patients with CCC are satisfactory if the patients undergo multidisciplinary treatment including chemotherapy and local radiation therapy prior to Ltx [9,10]. Due to the fact that the diagnosis of CCC can be extremely difficult to establish, PSC patients are usually evaluated for Ltx even when CCC is suspected and the final decision to perform transplantation is taken during laparotomy. If a limited tumour without extrahepatic tumour growth is found, the procedure should be continued and transplantation performed. Positive lymph nodes contraindicate transplantation, and the patient is given palliative therapy. Brandsaeter et al. reported that clinical suspicion of cancer, recent diagnosis of PSC and previous colon cancer are predictors of malignancy, which was found in 20% of more than 500 patients . Ideally, a PSC patient should be listed for Ltx at the time when survival with Ltx supersedes survival without Ltx. Prognostic models such as the Child-Pugh score or the model for end-stage liver disease (MELD) score have been demonstrated to be superior to PSC-specific prognostic models in predicting posttransplantation survival in PSC patients . Short duration of PSC, a high bilirubin and an elevated MELD score are associated with poor outcome . When assessing a PSC patient for Ltx a colonoscopy is mandatory. Patients with severe ulcerative colitis or mucosal dysplasia are candidates for colectomy before listing them for transplantation. Particular care is necessary to avoid life-threatening liver decompensation .
When reconstructing the biliary tract during transplantation two options are available. As experience with end-to-end biliary anastomosis is limited, most surgeons prefer a hepaticoenterostomy with a Roux-en-Y-loop in patients undergoing Ltx for PSC. There are a few reports that a duct-to-duct anastomosis is associated with a higher recurrence rate. From the pathophysiological point of view, neither alternative is logical since a possible source for malignancy remains untouched in situ. Whether an obligatory resection of the whole bile duct, meaning a pancreas head resection, is justified, has not yet been determined. Heightened attention is needed for the possibility of growth of malignant tissue infiltrating the choledochus duct. In this case, a simultaneous pancreatic head resection is generally accepted .
From the recipient's point of view and considering the development in waiting time in recent years, living related liver transplantation is the best option for PSC, not only in order to reduce waiting time but also to prevent patients from dropping off the list due to tumour formation . Patients with PSC in general have good physical status with low Child-Pugh score and therefore face a long waiting time. The risk of developing CCC correlates directly with the length of time the disease remains untreated.
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