J Pediatr 1976 Mar;88(3):434-8

The occurrence of hepatoma in the chronic form of hereditary tyrosinemia

Weinberg AG, Mize CE, Worthen HG

Abstract
A 5 1/2-year-old child with hepatocarcinoma complicating hereditary tyrosinemia
is presented. A review of the literature and an attempted follow-up of
previously reported patients with the chronic form of hereditary tyrosinemia
have disclosed 16 cases of hepatocarcinoma occurring in 43 patients surviving
beyond 2 years of age (37%). This incidence is considerably higher than that
generally given for the occurrence of hepatoma in adults with macronodular
cirrhosis. Females and males are equally at risk. Additional factors beyond the
development of cirrhosis are likely operative in the induction of
hepatocarcinoma in patients with this metabolic disorder; those surviving beyond
infancy are at considerable risk for the development of fatal hepatic neoplasms