Living related donor liver transplantation for primary sclerosing cholangitis with hepatocellular carcinoma and Crohn's disease: a case report.
BACKGROUND
Primary sclerosing cholangitis (PSC) is a premalignant condition associated with a risk of developing cholangiocarcinoma in 10% to 20% of patients. However, the prevalence of hepatocellular carcinoma (HCC) in patients with PSC is estimated to be only 2%. In addition, PSC often occurs in association with inflammatory bowel disease. Ulcerative colitis occurs in 60% of PSC patients and Crohn's disease occurs in about 10%. We diagnosed a patient as having PSC with HCC and concomitant Crohn's disease prior to living related donor liver transplantation (LRDLT).


METHODS
A 63-year-old woman was diagnosed as having PSC with solitary HCC. Preoperative liver condition was Child-Pugh grade C. Colonoscopic findings showed an active longitudinal ulcer in the terminal ileum and clinically diagnosed Crohn's disease. The medical treatment was 5'-aminosalicylates. She received a left lobe graft from her daughter. The immunosuppressants were tacrolimus and azathioprine, which was changed to predonisolone because of leukopenia and moderate acute cellular rejection, after which the postoperative course was uneventful. Colonoscopic findings revealed disappearance of the longitudinal ulcer in the terminal ileum at 15 months after LRDLT. There was no evidence of PSC or HCC recurrence.


CONCLUSIONS
Generally, PSC with HCC is rare with a poor prognosis; however, LRDLT may be a treatments option although the patient with PSC had a combined HCC. Moreover, aminosalicylates together with the immunosuppressants may be effective for the clinical management of concomitant Crohn's disease.
Oya
H
H
Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Sato
Y
Y
Yamamoto
S
S
Takeishi
T
T
Kobayashi
T
T
Hatakeyama
K
K
D015209:Cholangitis, Sclerosing / Q000150:complications / Q000601:surgery*; D003424:Crohn Disease / Q000150:complications / Q000601:surgery*; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008875:Middle Aged; D009679:Nuclear Family; D016896:Treatment Outcome