Rapidly progressing Budd-Chiari syndrome complicated by hepatocellular carcinoma.
Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.
Jang
Jeong Won
JW
Department of Internal Medicine, WHO Collaborating Center for Reference and Research on Viral Hepatitis, Catholic University of Korea College of Medicine, Seoul, Korea. garden@catholic.ac.kr
Yoon
Seung Kew
SK
Bae
Si Hyun
SH
Choi
Jong Young
JY
Chung
Kyu Won
KW
Sun
Hee Sik
HS
D000328:Adult; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D006528:Carcinoma, Hepatocellular / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology*; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D014057:Tomography, X-Ray Computed