Abstract
Background. Fibrocystic liver-kidney disease is caused by a group of rare and genetically diverse disorders that are associated with kidney cysts or dysplasia and ductal plate malformation in the liver. There have been several reports of liver neoplasias arising in hepatobiliary fibrocystic diseases. However, most were cholangiocarcinoma; cases involving hepatocellular carcinoma (HCC) are rare, and all the reported cases are related with adults.
Case Report. A 10-year-old girl with a history of repeated gastrointestinal bleeding underwent banding and sclerotherapy multiple times and had a history of a Portosystemic shunt without any significant benefit. She was referred to us as a case of fibrocystic liver disease with decompensated liver disease for liver transplantation. The patient underwent living donor liver transplantation, and the explanted liver histopathology report is documented. The explant liver weighed 838 g and measured 21 x 13 x 8.5 cm with the attached gallbladder measuring 7 x 3 x 0.2 cm (in wall thickness). The external surface was covered with multiple white nodules ranging in size from 0.4 to 1 cm. Serial slicing revealed an ill-defined, yellow, soft lesion (4 x 2.5 x 2.5 cm) localized in the subcapsular area of the left lobe (segment 4). The rest of the cut surface was green and nodular (cirrhotic). Microscopy from largest nodule was consistent with early hepatocellular carcinoma.The rest of the liver was cirrhotic, and the morphology was consistent with fibrocystic disease of liver.
Conclusion. We report a rare case of HCC associated with fibrocystic liver disease. When diagnosing fibrocystic liver disease without known risk factors, the presence of HCC must be considered, and vice versa. To our knowledge, this is the first reported case of HCC associated with fibrocystic liver disease in a 10-year-old child.