Hepatic masses constitute about 5-6% of all intra abdominal masses in children. Some of the common differential diagnosis of hepatic masses in children are: adenoma, hepatocellular carcinoma, focal nodular hyperplasia and hemangioma.
Hepatic mass also occure in children due to metabolic disorder.A 7-month-old infant was reffered to our ward due to developmental delay and unability to walk. Abdominal distention, hepato-splenomegaly, thickened wrists and failure to thrive (both height and weight were below 3 percentile) were the most features found in physical examination. He was the first child of first degree cousin parents without any significant medical history or previous hospitalization. The patient was admitted for further examination. After admission to the hospital, hypoglycemia occurred. On laboratory investigations, elevated aminotransferase, elevated alkalin phosphatase, hypocalcemia, hypophosphatemia and metabolic acidosis were obtained. Rachitic changes were noticeable in posteroanterior radiograph of his wrist. Increased liver size with heterogenous coarse parenchymal echogenicity associated with multiple variable size nodules were seen in abdominal sonography. A hypoecho solid well defined homogenous mass with posterior inhancement was located in left lobe of liver which was measured 45*38 mm. Splenomegaly with small and diffuse hypoecho nodular areas and Larg kidneys size with increased echogenicity were detected. With impression of metabolic disorder, alpha-fetoprotein and serum levels of aminoacid with HPLC method were performed. Elevated level of tyrosine level and alpha-fetoprotein (> 1000) magnified diagnosis. To confirm it, urine succinyl acetone was checked which was positive.MRI was performed to evaluate the hepatic mass and benign hepatic nodules were reported. The patient was treated with Nitisinone and restriction of tyrosine and phenylalanine in his diet. Outpatient follow up of hepatic mass was recommended.Hepatic masses are very rare in children. Metabolic disorder should be considered in hepatic masses. Although hepatic masses are well known in tyrosinemia, they accure in the ages more than 2 years, but delay in diagnosis of the disease can accompany with cirrhosis and hepatic adenoma in children less than 2 years old.