A 22-year-old man with thyroid cancer and cholestatic liver disease.

The diagnosis of PSC in association with ulcerative colitis was delayed in this case by the interim development of papillary carcinoma of the thyroid that produced a rapidly enlarging neck mass. The presence of chronic cholestasis, pruritus, and hepatosplenomegaly, along with a liver biopsy with histologic changes consistent with PSC, were not immediately appreciated because of the concern for the patient's thyroid cancer. In all other respects, the clinical features of PSC in this case are characteristic of the disease, with the somewhat unusual feature of intra-abdominal lymphadenopathy. Whether or not the papillary thyroid carcinoma is a coincidental finding rather than an associated disease entity, such as ulcerative colitis, remains open to speculation. In any event, the finding of papillary thyroid carcinoma or any other extrahepatic malignancy in association with PSC is rare, but assumes an added dimension for patients with PSC because the presence of malignancy may affect their suitability for future liver transplant.