Case 11.4

Labs checked during his flare showed a stably elevated alkaline phosphatase with no changes in his transaminases.

During the visit for his flare, the patient expressed concern about any malignancies associated with his diagnoses.

Which of the following are true regarding malignancies associated with PSC? (select all that apply)
Cholangiocarcinoma is unlikely to be present at the time of initial diagnosis of PSC
Cholangiocarcinoma can be the presenting feature of PSC or diagnosed within the first year of presentation in about 30% of patients.
Lifetime risk of developing cholangiocarcinoma in patients with PSC is 10-20%
PSC patients are at increased risk for developing cholangiocarcinoma. Although guidelines do not provide specific recommendations for screening for cholangiocarcinoma, most experts recommend MRCP with contrast MRI and CA 19-9.
Young age at the time of PSC diagnosis is associated with cholangiocarcinoma
Older age at PSC diagnosis, smoking, alcohol use, elevated bilirubin, a longer duration of IBD and dominant stricture are associated with a risk of CCA.
Ultrasound surveillance of gallbladder is recommended every year to evaluate for gallbladder polyp
PSC patients are at risk of developing gallbladder polyps, which can lead to cancer in 3% patients. Ultrasound surveillance of gallbladder is recommended every year. Cholecystectomy if polyps are >0.8 cm should be considered.
Patients with PSC and IBD are at two times increased risk of developing colon cancer as compared to IBD alone
Patients with PSC with IBD are at four times increased risk of developing colon cancer as compared to IBD alone. Colonoscopy with biopsies and/or chromoendoscopy for colon cancer surveillance is recommended every year even after liver transplantation.