Liver Transplantation for Non-resectable Perihilar Cholangiocarcinoma

Inclusion criteria * Radiologically strong suspicion of pCCA * Tumor can involve intrahepatic portal vein without thrombosis of extrahepatic portal vein * Tumor can involve hepatic artery distal to gastroduodenal artery or involve replaced hepatic artery * First time pCCA * Disease deemed not eligible for liver resection based on tumor location or underlying liver dysfunction * No extrahepatic disease, or lymph node involvement detected on imaging * No signs of extrahepatic metastatic disease according to PET-CT scan * No signs of extrahepatic metastatic disease according to CT or MR (chest/abdomen/pelvis) scan within 4 weeks prior to the faculty meeting at the transplant unit * At least 18 years of age * Good performance status, Eastern Cooperative Oncology Group (ECOG) score: 0 or 1 * Satisfactory blood tests Hb \>10g/dl, neutrophiles \>1.0 (after any G-CSF), TRC \>50, Bilirubin\<3 x upper normal level, ASAT, ALAT\<5 x upper normal level, Creatinine \<1.5 x upper normal level. Albumin above lower normal level, Normal IgG4 levels * Signed informed consent and expected cooperation of the patients for the treatment and follow up must be obtained and documented according to GCP, and national/local regulations * Received chemotherapy for at least 6 months with at least 10% response according ti RECIST criteria and with no progression of disease at time of Lt * At least 10 months from diagnosis * Patient must be accepted for transplantation before progressive disease * Twelve months or more time span from the diagnosis of pCCA and date of being listed for liver transplantation Exclusion criteria * Tumor involving common hepatic artery, celiac trunck or superior mesenteric artery the tumor * Tumor involving main portal vein * Tumor involving inferior vena cava * Perforation of the visceral peritoneum * Weight loss \>15% the last 6 months * Patient BMI \> 30 * Other malignancies, except curatively treated more than 5 years ago without relapse * Known history of human immunodeficiency virus (HIV) infection * Prior history of solid organ or bone marrow transplantation * Substance abuse, medical, psychological, or social conditions that may interfere with the patient's participation in the study or evaluation of the study results * Known hypersensitivity to rapamycin * Prior extrahepatic metastatic disease * Women who are pregnant or breast feeding * Any reason why, in the opinion of the investigator, the patient should not participate