
 hepatoblastoma is a primary malignant liver tumor that originates from embryonic and fetal liver cells .
it constitutes 30%-45% of all primary liver tumors and 50%-60% of all malignant tumors that occur during childhood.1 
 , 
 2 hepatoblastoma is often associated with a congenital anomaly , particularly with familial adenomatous polyposis coli .
it is primarily a tumor of young children , and more than 90% of tumors usually present before five years old , but rarely presents in older children or adults.4 , 
 5 males are affected twice as often as females .
however with increasing age , the gender difference equalizes . this is a relatively rapid growing tumor .
the serum alfa - fetoprotein ( afp ) levels are markedly elevated in approximately 90% of the patients .

 an 18-year - old man referred due to right upper quadrant ( ruq ) pain since two months prior to admission in this hospital .
the ruq pain , which was constant has had radiation to his back , and gradually was worsened .
he had complaints of low back pain , that aggravated by movement since one month prior to admission .
the patient had anorexia with a 15 kg weight loss during these two months with no history of fever , chills , vomiting , diarrhea , change in bowel habits or tarry stools . 
 

the liver was enlarged with firm two nodular consistency , and spleen was palpable by ballottement maneuver .
laboratory results were as follows : hemoglobin ( 9.1 g / dl ) , platelets ( 257000/mm ) , homocysteine : 20.5 mol / l ( normal 5 - 15 ) , anti - cardiolipinigm 2.9 mpl ( normal < 15 ) , anti - phospholipid igg : 1.8 ( normal < 10 ) , protein c : 127% ( normal 70 - 130 ) , protein s : 51% ( normal 65 - 140 ) , lupus anticoagulant : negative , alanine aminotransferase : 113 iu / l , aspartate aminotransferase : 495 iu / l , alkaline phosphatase : 1253
iu / l , total bilirubin : 12.6 mg / dl , direct bilirubin : 12 mg / dl and ldh : 789 u / l , serum hbs ag was positive and hbv dna serum level was 247 iu / ml . total protein , albumin , and coagulation indexes were within normal ranges .
paracentesis of ascitic fluid showed clear color with high serum ascites albumin gradient , without findings of peritonitis . 
 

ultrasonography of abdomen showed a coarse liver parenchyma , without mass or space occupying lesion .
endoscopy was notable for the presence of f1 esophageal varices , and fundal varices ( without active bleeding or stigmata of recent hemorrhage ) , and a kissing type duodenal ulcer disease . 
 

color doppler ultrasonography of abdomen showed splenic and portal vein thrombosis , but the hepatic artery and inferior vena cava were normal .
the initial serum alpha - fetoprotein level was 300 ng / ml ( normal 0 - 8.5 ) that after ten days increased to 112000 ng / ml .
triphasic ct scan of the liver shows a large heterogeneous caudate lobe , with small right and left liver lobes . 
 

there was thrombosis in the main portal vein with extension to the intra - hepatic branches , and splenic vein .
liver biopsy ( figs . 3 and 4 ) contained normal as well as neoplastic tissues . in the non - neoplastic tissues
the tumor component was composed of small and large nests of small hepatocyte - like cells .
the individual cells were round to polygonal shaped with amphophilic to somewhat eosinophilic cytoplasm and round to oval hyperchromatic nuclei with occasional nucleoli .
there were also scattered and large aggregates of polygonal cells with abundant fat accumulation in the cytoplasm ( fig .
3 ) . immunohistochemistry ( ihc ) was positive for hepar1 ( focal ) , vimentin , ck , afp , pcea and afp ( diffuse ) , and alfa-1 anti - trypsin .
ihc was negative for cd34 , synaptophysin , nse , ttf-1 , desmin , chromogranin , placental alkaline phosphatase , cd99 , cd10 , albumin , and s100 . according to the histopathology features and ihc profile ,
h&e stain of liver mass shows some fat vesicles with no evidence of cirrhosis . 
 


 hepatoblastoma is the most common primary liver tumor of childhood and adolescence , but is rarely found in adults , this tumor presents more commonly before the age of three years , predominantly in males.7- 
 9 however with increasing age the gender difference disappears and the incidence becomes equal among genders .
, hepatoblastoma can be presented with rapidly enlarging upper abdominal mass , weight loss , and or failure to thrive ; in adults symptoms of abdominal pain , fever , malaise and the presence of an abdominal mass are more frequent .
hepatocellular carcinoma is the most common primary liver tumor seen in adults and is closely related to cirrhosis and chronic liver disease , particularly in patients with chronic hepatitis b virus infection .
diagnosis of hepatocellular carcinoma should be considered in patients with cirrhosis who deteriorate clinically or has a liver mass in radiological workups . in any patient with cirrhosis and
newly developed signs of decompensation such as ascites , hepatic encephalopathy , variceal bleeding or fever of unknown origin , the presence of hepatocellular carcinoma should be investigated . 
 

our patient presented with ascites , esophageal varices and a large firm liver and positive hepatitis b virus antigen , so the initial clinical impression was hbv induced liver cirrhosis with hepatocellular carcinoma , but normal platelet count , and albumin was not in favor of advanced portal hypertension . 
 

hepatocellular carcinoma is the first diagnosis in any patient with clinical findings of cirrhosis , liver mass and elevated serum afp .
hcc have been reported in hbv infected non - cirrhotic young patients below age 20 but is a rare finding . in a report by sezaki et al . only four out of 187 patients with hcc were young with ages ; 10 , 22 , 23 , and 26 years .
they suggested predisposition to hcc due to integration of hbv genome into host genome during intrauterine life .
although development of portal vein thrombosis in a patient with cirrhosis can be explained , however the presence of both cirrhosis and hepatocellular carcinoma in a young adult with isolated hbv infection is very unusual . in young patients with liver mass ( es ) , without evidence of cirrhosis and an elevated afp , hepatoblastoma should be considered . 
 

the impression of caudate lobe hypertrophy in the radiologic reports can cause confusion in such cases for differentiation between cirrhosis plus hepatocellular carcinoma versus a large slowly growing hepatoblastoma arising from caudate lobe within non cirrhotic liver .
any pathology that causes caudate lobe enlargement can induce pressure effect on the portal venous system .
this process can induce right and left lobe liver atrophy and portal vein thrombosis also .
pathologic diagnosis of adult hepatoblastoma is difficult in that hepatic teratoma ; carcinosarcoma , malignant mesenchymal tumor , and hepatocellular with sarcomatous changes and hepatoblastomatous lesions should also be considered and be differentiated by appropriate immunohistochemistry evaluation . 
 

treatment of choice is complete surgical resection , and without surgery the survival is poor .
however improvements in survival have occurred with standardized chemotherapy that reduces the tumor size , enabling complete tumor excision . this process can be performed in patients who present with unresectable or metastatic disease .
chemotherapy reduces bleeding from the tumor and surrounding normal parenchyma and vascular structures , therefore facilitating resection of lesion .
chemotherapy has been proven effective as both an adjuvant and neoadjuvant treatment for tumor shrinkage , followed by surgical removal of the mass .
hepatoblastoma is sensitive to chemotherapeutic drugs such as doxorubicin , cisplatin , vincristine , 5-fu , and cyclophosphamide . 
 

this presentation of hepatoblastoma in a young patient with positive hepatitis b virus infection and portal hypertension ( ascites , esophageal varices , and splenomegaly ) is unusual .
however the normal platelet count and serum albumin in the patient is not in favor of advanced cirrhosis as the cause of portal hypertension .
the histologic evaluation of non - tumoral segments of liver can help us for differentiation of cirrhosis with hcc or a non - hcc liver tumor arising in a non - cirrhotic liver .
calcification within the mass , which is in favor of hepatoblastoma , was not shown in abdominal triphasic ct scan ; and only the liver biopsy results were positive for hepatoblastoma .
 a young man presented with a large liver mass and positive hepatitis b virus markers . 
 this 18-year - old male has developed ascites , jaundice , high serum alpha fetoprotein ( afp ) level , liver mass and portal hypertension , without fever or calcification in the mass . 
 all favored the diagnosis of rapidly , progressive hepatocellular carcinoma , however proven hepatoblastoma in liver biopsy . 
 hepatoblastoma usually manifests prior to the third year of life , but can rarely be seen in older children or adults . 
 although hcc rarely can be presented in young patients with hbv infection , but in patients without cirrhosis hepatoblastoma should be considered as the first possible diagnosis . 