A. Schlüter (Halle)  

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14 Years  

Female  

Abdomen-Liver  

neoplastic  

14 year-old female patient with a palpable tumor in the right hypochondrium at the point of diagnosis. Pain on pressure, icterus.  

This rare disease is observed predominantly in the 2nd and 3rd decade of life. In contrast to the hepatocellular carcinoma, no etiological connection to cirrhosis of the liver exists. Frequently, most solitary tumors already have a diameter of more than 10 cm in the initial diagnosis of the disease. Regressive variations are are frequent; in 30%, calcifications possible. Intrahepatic metastases as well as other organ metastases are are possible.  

<- view CT 1

CT 1: Spoke-wheel characteristic of the tumor (particularly after contrast application). Proof of central calcifications, as well as regressive tumor parenchymal changes. Inhomogenic contrast uptake in the right hepatic lobe when portal vessels are compressed.







<- view MRI 1

MRI 1: MRCP: Ectasias in the intrahepatic biliary duct because of tumor compression in the portal portion of the DHC.







<- view MRI 2

MRI 2: T2-coronal, Spoke-wheel characteristic of the tumor (particularly after contrast application). Proof of central calcifications, as well as regressive tumor parenchymal changes.







<- view MRI 3

MRI 3: T1-SE transversal, Spoke-wheel characteristic of the tumor (particularly after contrast application). Proof of central calcifications, as well as regressive tumor parenchymal changes. Inhomogenic contrast uptake in the right hepatic lobe when portal vessels are compressed.




 

Surgery / Histo  

The whole constellation - particularly the proof of calcifications as well as the wheel-spoke character of the lesions - are typical for the fibrolamellar liver carcinoma. Differentials which should be considered are benign tumors (FNH, Adenoma).  

The illness is rare. The diagnosis is often made in late stages of the illness. Mostly a solitary finding. Metastases are possible. The prognosis is principally not good. It is dependent on: if the tumor is able to be resected and if metastases are found. Reoccurrances have been described after surgical intervention. Local or systemic chemotherapy increase the tumor-free intervals.  

N/A  

N/A  

1. Medline:
Ichikawa T, Federle M P, Grazioli L, Marsh W.
Fibrolamellar hepatocellular carcinoma: pre- and posttherapy evaluation with CT and MR imaging.
Radiology 2000; 217, 145-151  

Fibrolamellaric liver carcinoma, liver tumor, Fibrolamellar liver carcinoma, abdominal tumor, pain, icterus, child, childhood, pediatric radiology  

A Schlüter. Fibrolamellar liver carcinoma in the childhood. PedRad [serial online] vol 1, no. 11.
URL: www.PedRad.info/?search=20011128193914