Budd-Chiari Syndrome by Xingshun Qi
Author:Xingshun Qi
Language: eng
Format: epub, pdf
ISBN: 9789813292321
Publisher: Springer Singapore
9.4 Treatment
Barcelona group guidelines have been established to standardize the management of HCC occurring in the setting of adults with cirrhosis. Although in practice patients with BCS-HCC are routinely managed according to these guidelines, as HCC usually occurs on cirrhosis, there is no evidence that they could be applied when the underlying liver disease is a BCS.
The modified Barcelona Clinic Liver Cancer (BCLC) staging system and treatment strategy [33] clearly stated that “preserved liver function” refers to Child-Pugh class A without any ascites. However, in BCS, ascites may occur in a patient with well-preserved liver function, and with almost normal prothrombin time and platelet count. In this case, a surgical resection or another treatment could be performed after removal of the ascites by a TIPS placement. In the same way, if a BCS patient had multiple hypervascular nodules with no characteristic washout in the portal phases, and a high AFP level, even if only one lesion is an HCC, we will consider that this patient is at an intermediate-stage (BCLC-B: multinodular asymptomatic tumors without vascular invasion or extrahepatic spread), and thus the first-line choice therapy should be TACE, while the patient is in fact BCLC-A and should rather benefit from a LT. Many questions remain about therapeutic management, mainly because of the small sample size of published studies, and a lack of evidence. Since there is no generally accepted treatment recommendation, the management of BCS-associated HCC should be discussed in a multidisciplinary meeting.
Several therapeutic options have been used to treat BCS-HCC, including surgical resection, LT, TACE, or local ablative therapies. Venous drainage should be associated with the treatment of HCC, since restoration of hepatic venous drainage may reduce the risk of HCC occurrence and probably recurrence [7, 8], but at present, there is no consensus regarding whether these drainage modalities should be implemented before or after HCC treatment.
Tumor resection is a curative treatment. However, some authors do not rank it as a favored option in the setting of BCS, because of its high morbidity and mortality compared to surgery for HCC related to other etiologies [38, 39]. Previous procedures on IVC and HV, and the development of subcapsular collateral veins could complicate liver surgery. On the contrary, other authors reported good results after HCC surgical treatment, especially when it was possible to treat HVOO and HCC in the same time or sequentially, with a reduced incidence of complications and extended survival. Thirty-eight patients with BCS-HCC association underwent liver resection, and 22 of them benefited from cavo-atrial shunt to remove HVOO in addition to liver resection. The combined surgery group had a significantly longer survival and a lower incidence of post-operative complications compared to the liver resection group: 9.1% versus 37.5%. HVOO relief was a protective factor for survival of patients with BCS-HCC in this study [40]. Based on clinical data, if a liver resection is decided, it is better to schedule it as soon as possible after the restoration of hepatic venous outflow, as this would decrease liver congestion and bleeding complication during the intervention [37].
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