Livertumor in a Patient with Beta-Thalassemia maior (BTM) – a case report

Problemstellung: In a 40- year- old Iranian woman a slightly echogenic liver tumor had been detected several years ago. The type of vascularisation resembled a focal nodular hyperplasia. On contrast-enhanced ultrasound (Sonovue (Bracco), GE Logiq E9) no wash-out was observed. Patienten und Methode: The patient had been suffering from BTM with the consequence of iatrogenic hemosiderosis due to regular blood transfusions. She was negative for hepatitis B and C. On close follow up the size of the liver lesion was stable for five years, when slight growth and a wash-out of contrast agent was observed. A fine-needle puncture revealed malignancy and a left lateral liver resection was performed. Ergebnisse: The tumor was characterized as intrahepatic cholangiocellular carcinoma within an advanced liver fibrosis with significant secondary siderosis (pT1pN0). Luckily the post operative course was uneventful and one year clinical and sonographical follow-up showed no further tumor growth. Schlussfolgerungen: As a result of improved survival, hepatocellular carcinoma (HCC) has emerged in thalassemics only recently. Due to blood transfusions these patients are often infected with hepatitis C or B virus and often have a siderosis of the liver. There are few well-established risk factors for cholangiocarcinoma (CC), including primary sclerosing cholangitis, liver fluke infestations, hepatolithiasis, Thorotrast and choledochal cysts. CC in hemosiderosis seems rare, but a prevalence of beta-thalassemia and cholangiocarcinoma is known for Thailand. Our case of a long follow-up without malignant growth favours the evidence that development of CC involves a multiple-step progression from a benign to a malignant nodule.