G. Hahn (Bereich Kinderradiologie/Uniklinikum Dresden/Deutschland)  

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6 Months  

Female  

Abdomen-Liver  

neoplastic  

In a routine sonographical examination in a child with previously known Beckwith-Wiedemann Syndrome, a large tumor of the liver was found. Proof of slight scleral jaundice. Markedly increased alpha-fetoprotein in the blood.  

Hepatoblastomas is a malignant embryonal mixed tumor, which derives from epithelial and/or mesechymal cellular elements. It occurs in early childhood.
In Beckwith-Wiedemann Syndrome, one findes more commonly nephroblastomas and hepatoblastomas.
In increased alpha-fetoprotein in the blood and proof of a typical hepatic tumor in MRI, the diagnosis is relatively easy and there is no histological proof needed before starting chemotherapy.  

<- view MRI 1

MRI 1: T2 transverse: Inhomogenic, partly focally hyperintense and partly hypointense, mass of ca. 9 x 7 x 12 cm in size in the right and left hepatic lobe. The vena portae is considerably compressed ventrally and is pushed to the left, as is the pancreas. The gall bladder is no longer demarcated.
The right kidney is compressed from above and thus has moved caudally. The vena cava's lumen is reduced to a slit due to compression in the region of the tumor.




<- view MRI 2

MRI 2: T2 coronal: Inhomogenic, partly focally hyperintense and partly hypointense, mass of ca. 9 x 7 x 12 cm in size in the right and left hepatic lobe.




<- view MRI 3

MRI 3: T1 transversal: Inhomogenic, partly focally hyperintense and partly hypointense, mass of ca. 9 x 7 x 12 cm in size in the right and left hepatic lobe.The right kidney is compressed from above and thus has moved caudally.




<- view MRI 4

MRI 4: T1 sagittal, contrast media, fat saturation: After contrast media application, inhomogenic enhancement of the intrahepatic mass, which in the T1-sequence allows delimination of focally hyperintense masses of only a few millimeters.




<- view MRI 5

MRI 5: T1 coronar, contrast media, fat saturation: After contrast media application, inhomogenic enhancement of the intrahepatic mass, which in the T1-sequence allows delimination of focally hyperintense masses of only a few millimeters.




<- view MRI 6

MRI 6: T1 transversal, contrast media, fat saturation: After contrast media application, inhomogenic enhancement of the intrahepatic mass, which in the T1-sequence allows delimination of focally hyperintense masses of only a few millimeters.



 

Surgery / Histo  

Hepatocellular carcinoma, in childhood rare.  

In good size reduction through chemotherapy and following complete surgical resection, the prognosis is very good.  

N/A  

N/A  

N/A  

Beckwith-Wiedemann Syndrome, Hepatoblastoma, malignant embryonal mixed tumor, child, childhood, pediatric radiology  

G Hahn. Hepatoblastoma. PedRad [serial online] vol 4, no. 12.
URL: www.PedRad.info/?search=20041222171655