HEPATIC ADENOMA IN THE PEDIATRIC AGE GROUP - CLINICOPATHOLOGICAL OBSERVATIONS AND ASSESSMENT OF CELL PROLIFERATIVE ACTIVITY

The clinicopathological findings of eight children with hepatic adenom a in the absence of cirrhosis are presented. The lesions ranged in dia meter from 0.1 to 14.5 cm. Associated disorders were Fanconi's anemia, type I glycogen storage disease, Hurler's disease, and severe combine d immunodeficiency with ADA deficiency. The remaining three children h ad adenoma without known associated disorders. In the children with gl ycogenosis and Hurler's disease the adenomas were multiple. Significan t dysplasia occurred in the two children with Fanconi's anemia; howeve r, the lesions behaved in a benign fashion-one with regression of the tumor after cessation of androgen therapy and the other with nonrecurr ence after complete resection. Proliferating cell nuclear antigen (PCN A) labeling index (LI) of the adenoma arising in patients with Fanconi 's anemia was significantly greater than the PCNA-LI of adenoma in the other children (mean 4.1% versus 0.9% of nuclei), approaching the low er end of the spectrum for reported hepatocellular carcinoma cases. We emphasize that the worrisome pathology that may occur in hepatic aden oma in children, particularly with Fanconi's anemia, does not necessar ily pre diet malignant behavior. The association of hepatic adenoma wi th Hurler's disease or severe combined immunodeficiency has not been r eported previously.