Well-Differentiated Hepatocellular Neoplasms in Adult Women with Congenital Hepatic Fibrosis
DW Azar, A Shabaik, MR Peterson. University of California, San Diego, La Jolla, CA
Background: Congenital hepatic fibrosis (CHF) is a genetic disorder associated with autosomal recessive polycystic kidney disease (ARPKD), characterized by portal based fibrosis and anastomosing bile ducts, and leading to hepatomegaly and portal hypertension. Rare cases of hepatic mass lesions including adenomatous hyperplasia (1 case), hepatocellular carcinoma (1 case), and dysplastic nodule (1 case) have been reported in patients with CHF. We here report two cases of well differentiated hepatocellular neoplasms with features intermediate between adenoma and hepatocellular carcinoma arrising in adult women with CHF.
Design: The first patient was a 22 year old female with known ARPKD in whom two hepatic masses were identified via routine screening, a 3.4 cm segment II mass and a 5.2 cm right lobe mass. The second patient was a 34 year old female presenting with asymptomatic AST/ALT elevations found to have an 8.5 cm mass in the caudate lobe. Neither patient was taking oral contraceptives at the time of diagnosis. Both patients underwent partial hepatectomy.
Results: Features consistent with CHF were confirmed in non-neoplastic liver parenchyma in both patients. Both resected tumors were characterized by sheets of uniform, well differentiated hepatocytes with low N/C ratios and low mitotic indices. The second tumor was also extensively steatotic. Wide fibrous bands extended through both tumors, but biliary structures were entirely absent from the mass lesions. Focal areas of large-cell dysplasia were identified in the second tumor. Reticulin staining showed areas of both tumors with loss of reticulin fibers. CD34 immunostaining on both tumors revealed patchy sinusoidal positivity, in contrast to the background liver parenchyma. Glypican-3 immunostaining on the second tumor showed faint granular cytoplasmic reactivity.
Conclusions: We present two cases of well-differentiated hepatocellular neoplasms developing in young adult female patients with congenital hepatic fibrosis. While both tumors exhibited features of hepatic adenoma, the clinical presentations are atypical for hepatic adenoma and both demonstrated atypical features, including areas of reticulin framework loss, CD34 sinusoidal positivity, and large cell dysplasia (one case). These cases add to the limited reports of hepatocellular neoplasms arising in CHF. While the atypical features raise concern for malignancy, only long-term follow-up and expanded case volume will attest to the prognosis of such lesions.
Category: Liver & Pancreas
Tuesday, March 23, 2010 9:30 AM
Poster Session III # 225, Tuesday Morning