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656 LIVER MALIGNANCY FOLLOWING PROLONGED PARENTERAL NUTRITION (PN)

Cholestatic jaundice (ChJ) is a well known and presumably benign complication of prolonged PN. We now report a preterm infant in whom fatal hepatocellular carcinoma (CA) occurred as a consequence of biliary cirrhosis secondary to prolonged PN and PN-associated ChJ. The patient was a 900 gram, 27-week gestation infant. Recurrent episodes of necrotizing enterocolitis led to small bowel resection and resultant short gut syndrome, necessitating PN for a total of 395 days. During this time progressive liver dysfunction developed, with marked conjugated hyperbili-rubinemia (maximum 11.2 mg/dl), and increased transaminase levels. Liver biopsy after 112 days of PN revealed periportal fibrosis and intracellular cholestasis. All liver function tests were normal when discharged at age 19 months. He was readmitted six months later with fever, hepatomegaly, icterus, and melanotic stools, and expired after the onset of massive GI hemorrhage. At autopsy, two separate hepatic tumor masses were found, one of which had invaded the posterior wall of the duodenum. Microscopically, the histology was that of hepatocellular CA; the tumor was composed of neoplastic hepatocytes arranged in trabeculae or lying in non-cohesive masses, with definite cannicular bile stasis and no hematopoietic tissue or mesenchymal components. All non-tumor areas of the liver showed biliary cirrhosis. In summary, this patient demonstrates the oncogenic potential of biliary cirrhosis complicating PN-associated liver dysfunction.