Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performin...Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation(LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma(HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation AssociationEuropean Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma(HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient.Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches,alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.展开更多
We presented a case of chronic recurrent hepatic encephalopathy occurring in a liver cirrhosis patient (Child Pugh A) with a large gastrorenal shunt and a review of the literature focusing on diagnosis and management....We presented a case of chronic recurrent hepatic encephalopathy occurring in a liver cirrhosis patient (Child Pugh A) with a large gastrorenal shunt and a review of the literature focusing on diagnosis and management. Computed tomography (CT) demonstrated an atrophic liver, splenomegaly, varices at the gastric fundic and the splenic hilum, and a highly tortuous shunt vessel between the gastric fundic varices and the left renal vein. Ultrasonography revealed the portal vein diameter was 0.8 cm; and portal vein blood flow velocity was 17.6 mL/s. In practice, the clinician need to ascertain the existence of the large portosystemic shunt when the patients presenting hepatic encephalopathy recurrent or refractory to standard medical treatments.展开更多
文摘Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation(LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma(HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation AssociationEuropean Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma(HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient.Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches,alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.
基金Supported by National Natural Science Foundation of China ( 30900671 ) and Shanghai Natural Science Foundation,China(09ZR1419700)
文摘We presented a case of chronic recurrent hepatic encephalopathy occurring in a liver cirrhosis patient (Child Pugh A) with a large gastrorenal shunt and a review of the literature focusing on diagnosis and management. Computed tomography (CT) demonstrated an atrophic liver, splenomegaly, varices at the gastric fundic and the splenic hilum, and a highly tortuous shunt vessel between the gastric fundic varices and the left renal vein. Ultrasonography revealed the portal vein diameter was 0.8 cm; and portal vein blood flow velocity was 17.6 mL/s. In practice, the clinician need to ascertain the existence of the large portosystemic shunt when the patients presenting hepatic encephalopathy recurrent or refractory to standard medical treatments.
