A pooled analysis of treatment and prognosis of hepatic angiosarcomain adults

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.

Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome

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.

Primary hepatic angiosarcoma manifesting as hepatic sinusoidal obstruction syndrome:A case report

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.

Malignant hepatic vascular tumors in adults: Characteristics,diagnostic difficulties and current management

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.

Primary hepatic angiosarcoma: A case report

BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignant tumor of the vascular endothelium.Clinical manifestations and laboratory and imaging examinations often lack specificity for PHA.We report a case of PHA,and describe the ultrasound characteristics and characteristic changes in laboratory values associated with PHA.CASE SUMMARY A 75-year-old woman presented with right upper quadrant abdominal pain for half a month.Magnetic resonance imaging(MRI)at a local hospital revealed multiple liver space-occupying lesions,and she was admitted to our hospital for further diagnosis.Contrast-enhanced ultrasound(CEUS)revealed multiple slightly hyperechoic nodules in the liver,which were suspected to be of malignant vascular origin.Contrast-enhanced computed tomography revealed multiple lowdensity nodules in the liver,considered to be metastatic hematopoietic malignancies.Contrast-enhanced MRI showed that the multiple liver nodules shared features with infectious lesions.Laboratory examination revealed normal alpha-fetoprotein levels,slightly increased other liver enzymes,decreased platelets,and significantly increased D-dimer levels.Liver biopsy and histopathology confirmed the presence of PHA.CONCLUSION CEUS can provide valuable clues for the diagnosis of PHA and greatly improve the success rate of puncture biopsy.

Hepatic angiosarcoma:an analysis of 7 cases

Objective： The aim of our study was to evaluate and sum up the experience with hepatic angiosarcoma. Methods： Retrospective study of 7 patients with hepatic angiosarcoma. Results： Symptoms and signs such as abdominal pain were extremely nonspecific. The levels of the tumor markers were within the normal range. Four cases were positive for hepatitis B viruses. Among the 5 patients who received chemotherapy, improvement in survival was seen in 3 cases which also received surgery. Conclusion： Hepatic angiosarcoma is a rare and diffficult-to-diagnose malignant liver tumor. Surgery is the definitive treatment and adjuvant chemotherapy after surgery may improve survival.

A complementary comment on primary hepatic angiosarcoma:A case report

BACKGROUND This article examines primary hepatic angiosarcoma(PHA)and fat-poor angiomyolipoma(AML),two uncommon vascular cancers.Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques.Uncommon malignant tumors of the vascular endothelium include PHA.Another diagnosis that should not be overlooked when employing contrastenhanced MR and contrast-enhanced computed tomography(CT)imaging techniques is fat-poor AML,one of the uncommon vascular tumors of the liver.In both conditions,biopsy is the primary means of diagnosis.CASE SUMMARY In our article,besides the diagnosis of PHA,fat-poor AML,one of the other rare vascular tumors of the liver,is mentioned.In the case,a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain,weight loss,and nausea.Abdominal ultrasonography(US)revealed a hypoechoic heterogeneous lesion with occasional faint contours.In computed tomography,it was observed as a hyperdense nodular lesion in segment 4.Magnetic resonance imaging(MRI)revealed that the lesion did not contain fat.In connection with the known history of VHL Syndrome,we first evaluated the possibility of AML.Thereupon,a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5%fat content.CONCLUSION In conclusion,PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences.Important imaging techniques like contrast-enhanced US(CEUS),CECT,and CEMRI give us substantial advantages in both cases.However,a biopsy is used to provide the final diagnosis.

Rapidly Progressing Hepatic Angiosarcoma Terminally Cared for at Home: A Case Discussion—Hepatic Angiosarcoma Taken Palliative Care at Home

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.

Pediatric vascular tumors of the liver:Review from the pathologist’s point of view

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.

Primary malignant vascular tumors of the liver in children:Angiosarcoma and epithelioid hemangioendothelioma

Primary malignant vascular neoplasms of the liver,angiosarcoma and epithelioid hemangioendothelioma,are extremely rare entities in the pediatric population.International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group.In this article,we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population.Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis.The distinct histologic features include whorls of atypical spindled cells and eosinophilic globules,in addition to the general findings of angiosarcoma.Histologic diagnosis of pediatric hepatic angiosarcoma is not always straightforward,and the diagnostic challenges are discussed in the article.Hepatic epithelioid hemangioendothelioma also demonstrates a female predominance,but is more commonly identified in adolescents(median age at diagnosis:12 years).Histologically,the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid stroma.While WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma,there are currently no known signature genetic alterations seen in pediatric hepatic angiosarcoma.Advancement in molecular pathology,particularly for pediatric hepatic angiosarcoma,is necessary for a better understanding of the disease biology,diagnosis,and development of targeted therapies.