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
Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A sys...Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A systematic literature search was conducted using PubMed, Embase and Chinese BiomedicalLiterature database, to identify articles published from January 1980 to July 2017. Search termswere “hepatic angiosarcoma” and “liver angiosarcoma”. Additional articles were retrieved through manualsearch of bibliographies of the relevant articles. Pooled individual data concerning the prognosis followingvarious therapeutic modalities were analyzed.展开更多
Differential diagnosis of pediatric vascular liver tumors can be challenging due to inconsistent nomenclature,histologic overlap and the rarity of some entities.Here we give an up-to-date overview of the most importan...Differential diagnosis of pediatric vascular liver tumors can be challenging due to inconsistent nomenclature,histologic overlap and the rarity of some entities.Here we give an up-to-date overview of the most important entities.We discuss the clinic,histology and pathophysiology of hepatic congenital and infantile heman-gioma,hepatic epithelioid hemangioendothelioma and hepatic angio-sarcoma.展开更多
Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulate...Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome(KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.展开更多
Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with...Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.展开更多
Background: Hepatic angiosarcoma is a rare disease with a poor prognosis due to its tendency for distant sites. Few opportunities exist for palliative treatment of hepatic angiosarcomas at home. Here, we report a rare...Background: Hepatic angiosarcoma is a rare disease with a poor prognosis due to its tendency for distant sites. Few opportunities exist for palliative treatment of hepatic angiosarcomas at home. Here, we report a rare case of palliative treatment of hepatic angiosarcoma at home. Case Presentation: An 87-year-old male patient complained of upper abdominal pain and anorexia, persisting for 2 months. Computed tomography revealed multiple tumors in the liver, spleen, left kidney, and bone. Hepatic angiosarcoma was diagnosed based on the liver biopsy results. Due to his advanced age and dementia, the patient and his family decided to receive palliative treatment at home, thereby initiating home medical care at our clinic. During the first visit, oral opioid medication was introduced, and home oxygen therapy (HOT) was initiated because of complaints of cancer pain and respiratory distress. As oral intake became difficult, the patient was switched to a patch opioid, and suppositories were used for ton use. The patient was treated with morphine and HOT;however, the improvement in respiratory distress was below acceptable. The patient died at home on the ninth day after his visit. Conclusion: To the best of our knowledge, this is the first case report on palliative care for hepatic angiosarcoma at home. Owing to the rapid progression of this disease, home physicians must know its characteristics and provide appropriate medical care.展开更多
BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a hig...BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.展开更多
1病例资料患者男,49岁,因“间断性上腹部疼痛不适10余天”于2020年7月3日入院。患者既往体健,无特殊病史,无化学毒物及放射性物质接触史。入院后,体格检查示右上腹部轻度压痛,余未见明显阳性体征。实验室检查结果示乙型肝炎表面抗原(hep...1病例资料患者男,49岁,因“间断性上腹部疼痛不适10余天”于2020年7月3日入院。患者既往体健,无特殊病史,无化学毒物及放射性物质接触史。入院后,体格检查示右上腹部轻度压痛,余未见明显阳性体征。实验室检查结果示乙型肝炎表面抗原(hepatitis B surface antigen,HBs Ag)、乙型肝炎表面抗体(hepatitis B surface antibody,HBs Ab)、乙型肝炎e抗原(hepatitis B e antigen,HBe Ag)、乙型肝炎核心抗体(hepatitis B core antibody,HBc Ab)均为阳性,HBV DNA为8.14×10^(5)IU/m L,血小板计数为146×10^(9)/L,甲胎蛋白为5.69 ng/m L,其他生化指标未见明显异常。展开更多
Background: Hepatic angiosarcoma is a rare tumor of endothelial origin that accounts for up to 2% of all primary neoplasms of the liver. It is uncommon in young adults and acute liver failure is a very unusual present...Background: Hepatic angiosarcoma is a rare tumor of endothelial origin that accounts for up to 2% of all primary neoplasms of the liver. It is uncommon in young adults and acute liver failure is a very unusual presentation of this disease. Case Presentation: We report the cases of two young male adults who presented with acute liver failure. One of them was diagnosed with primary hepatic angiosarcoma after transplantation based on the complete evaluation of the heaptectomy specimen;while the other was diagnosed through a liver biopsy which was done as part of the work-up for transplantation. Both patients had a rapidly worsening clinical course and died within 2 months of diagnosis. Conclusion: Hepatic angiosarcoma is a poor prognosis disease with high and early mortality. Therefore, a high level of suspicion should be present to promptly diagnose it, especially when dealing with patients with a rapidly worsening liver disease.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.
文摘Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A systematic literature search was conducted using PubMed, Embase and Chinese BiomedicalLiterature database, to identify articles published from January 1980 to July 2017. Search termswere “hepatic angiosarcoma” and “liver angiosarcoma”. Additional articles were retrieved through manualsearch of bibliographies of the relevant articles. Pooled individual data concerning the prognosis followingvarious therapeutic modalities were analyzed.
文摘Differential diagnosis of pediatric vascular liver tumors can be challenging due to inconsistent nomenclature,histologic overlap and the rarity of some entities.Here we give an up-to-date overview of the most important entities.We discuss the clinic,histology and pathophysiology of hepatic congenital and infantile heman-gioma,hepatic epithelioid hemangioendothelioma and hepatic angio-sarcoma.
文摘Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome(KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.
文摘Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.
文摘Background: Hepatic angiosarcoma is a rare disease with a poor prognosis due to its tendency for distant sites. Few opportunities exist for palliative treatment of hepatic angiosarcomas at home. Here, we report a rare case of palliative treatment of hepatic angiosarcoma at home. Case Presentation: An 87-year-old male patient complained of upper abdominal pain and anorexia, persisting for 2 months. Computed tomography revealed multiple tumors in the liver, spleen, left kidney, and bone. Hepatic angiosarcoma was diagnosed based on the liver biopsy results. Due to his advanced age and dementia, the patient and his family decided to receive palliative treatment at home, thereby initiating home medical care at our clinic. During the first visit, oral opioid medication was introduced, and home oxygen therapy (HOT) was initiated because of complaints of cancer pain and respiratory distress. As oral intake became difficult, the patient was switched to a patch opioid, and suppositories were used for ton use. The patient was treated with morphine and HOT;however, the improvement in respiratory distress was below acceptable. The patient died at home on the ninth day after his visit. Conclusion: To the best of our knowledge, this is the first case report on palliative care for hepatic angiosarcoma at home. Owing to the rapid progression of this disease, home physicians must know its characteristics and provide appropriate medical care.
基金Supported by Tianjin Science and Technology Plan Project,No.19ZXDBSY00030Beijing iGandan Foundation,No.RGGJJ-2021-014.
文摘BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.
文摘1病例资料患者男,49岁,因“间断性上腹部疼痛不适10余天”于2020年7月3日入院。患者既往体健,无特殊病史,无化学毒物及放射性物质接触史。入院后,体格检查示右上腹部轻度压痛,余未见明显阳性体征。实验室检查结果示乙型肝炎表面抗原(hepatitis B surface antigen,HBs Ag)、乙型肝炎表面抗体(hepatitis B surface antibody,HBs Ab)、乙型肝炎e抗原(hepatitis B e antigen,HBe Ag)、乙型肝炎核心抗体(hepatitis B core antibody,HBc Ab)均为阳性,HBV DNA为8.14×10^(5)IU/m L,血小板计数为146×10^(9)/L,甲胎蛋白为5.69 ng/m L,其他生化指标未见明显异常。
文摘Background: Hepatic angiosarcoma is a rare tumor of endothelial origin that accounts for up to 2% of all primary neoplasms of the liver. It is uncommon in young adults and acute liver failure is a very unusual presentation of this disease. Case Presentation: We report the cases of two young male adults who presented with acute liver failure. One of them was diagnosed with primary hepatic angiosarcoma after transplantation based on the complete evaluation of the heaptectomy specimen;while the other was diagnosed through a liver biopsy which was done as part of the work-up for transplantation. Both patients had a rapidly worsening clinical course and died within 2 months of diagnosis. Conclusion: Hepatic angiosarcoma is a poor prognosis disease with high and early mortality. Therefore, a high level of suspicion should be present to promptly diagnose it, especially when dealing with patients with a rapidly worsening liver disease.
