Peculiar Hepatocellular Carcinoma Clinical Findings in patients with Thalassemia Syndromes.

Abstract

Abstract 5108 BackgroundHepatocellular carcinoma (HCC) is the most common primary hepatic cancer, and the fifth and eighth most common malignancy worldwide in men and women, respectively. Hepatitis B and C, primary and secondary hemochromatosis and cirrhosis have been identified as major risk factors. Hepatitis C or B virus infection and secondary hemochromatosis are the major cause of morbidity and mortality. In the last years, HCC on cirrhosis is a common reported complication in patients with thalassemia syndromes1,2,3. In non thalassemia patients HCC is a late complication of cirrhosis and prognosis depends on suitability for treatment. In this group, the last is low (40%) considering that cirrhosis severe stage C, according to Child-Pugh score is generally observed (personal communication). Aim of the studydescribing, in a homogeneous cohort of thalassemia patients, the main clinical findings of HCC. Patients and MethodsAll subjects with risk factors for HCC as iron overloading, HCV or HBV chronic infection, histological evidence of cirrhosis were undergone every six months to an ultrasound evaluation associated with alphaphetoprotein determination. Nine new cases, since January 2000 until July 2009, were detected. Table I shows the main clinical findings. Mean value of serum ferritin levels, in the last 2 years before diagnosis, was 1049±721. All patients except one were anti-HCV positive (Table I). Seven of them were HCV-RNA positive (Table I). None of them had previous HBV infection. ResultsOnly one patient had severe stage C, according to Child-Pugh score. None of the patients had significant high levels of alphaphetoprotein (AFP) at diagnosis. However, a slightly increase of AFP levels was shown, during the 6 months before diagnosis, in 50% of cases. Three patients had a delayed diagnosis because of atypical imaging for HCC at TC scan or a negative previous histology for HCC after liver biopsy. Five patients had multifocal HCC at the diagnosis (Table I). Four showed an unifocal HCC, developing two of them multifocal HCC during the follow-up (Table I). Four patients died during the follow-up for decompensated cirrhosis. Five patients are alive after treatment. Main used treatments are shown on Table I. The overall mean survival was 28±25 months (range 3-64). Conclusions1) almost all talassemia patients with HCC are suitable for treatment ; 2) baseline liver function is the most main factor conditioning survival and suitability for treatment ; 3) periodical serum AFP determnations can reveal a slight increase; 3) iron overloading alone can be related to the development of HCC in a liver with still good functional reserve, giving more opportunity for treatment 4) monitoring for HCC by liver ultrasound evaluation, every six months, in at risk patients with thalassemia syndromes should be mandatory.Table IMain clinical findings of HCC in thalassemia syndromes.PATIENTSSEXPHENO-TYPEAGESURVIVAL FROM DIAGNOSIS (MONTHS)DEADHCV ABHCV RNAUNIFOCAL OR MULTI-FOCAL AT DIAGNOSISFERRITIN (ng/ml)THERAPY1FTM546-++M890TACE2MTM4360+++M1.036TACE3FTI5612+++M1.065TACE SORAFENIB4FTI5923-+-U990TACE5MTI4358-OLT-++U>M2.124RFTA TACE OLT6MTI4818+++M2.262-7FTI6864-++U>M310RESECTION PEI TACE8MTI735---U574PEI TACE9MTM503+++M194SORAFENIBTI: Thalassemia Intermedia; TM: Thalassemia Major; OLT: Orthotopic Liver Transplantation; U: Unifocal; M: Multifocal;Ferritin: mean values in the last 2 yearsPEI: Percutaneous Ethanol Injection; TACE:Transarterial Chemoembolization; RFTA:Radiofrequency Thermal Ablation DisclosuresNo relevant conflicts of interest to declare.