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Hepatic angiosarcoma is a rare disease, and hepatic hemangiosarcoma with Kasabach-Merritt syndrome (KMS) is even rarer. Although there have been several reports about KMS caused by hepatic angiosarcoma, there has been no mention of successful treatment regimens for hepatic angiosarcoma with KMS. A 64-year-old female patient presented with right upper abdominal pain and multiple cutaneous purpuras for 10 days. Blood analysis revealed that hemoglobin, platelet and fibrinogen were significantly decreased, prothrombin time was prolonged, fibrinogen degradation products were increased. Contrast-enhanced computed tomography scan of the abdomen demonstrated a large mass in the right lobe of the liver, which is pathologically suggestive of hepatic angiosarcoma. Based on the above examination, the patient was diagnosed with KMS caused by hepatic angiosarcoma. Repeated transfusion of blood products could only temporarily improve the coagulation function of the patient. After transarterial chemoembolization, the patient experienced a long-term improvement of blood clotting, and the patient's survival increased by six months. Transarterial chemoembolization should be considered one of effective therapies for hepatic angiosarcoma with KMS.

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.

Hepatic angiosarcoma is a rare, highly aggressive mesenchymal liver malignancy with poor prognosis that stems from the endothelial cells that line the walls of blood or lymphatic vessels. It is the third most common primary liver malignancy and is most prevalent among older males. It is difficult to diagnose due to various clinical presentations from asymptomatic to abdominal pain, pleural effusion, and liver failure. The diagnosis of liver angiosarcoma is suspected on imaging features and confirmed by histopathological assessment. Primary management is determined based on the stage of tumor from surgery to palliative care such as chemotherapy or tumor transarterial embolization. We report a 51-year-old female who presented with stage 4 liver angiosarcoma and a history of childhood Wilms tumor. We focus on tumor management using radiological modalities and pathological analysis and discuss secondary liver tumors in survivors of childhood Wilms tumor.

Background Primary hepatic angiosarcoma (PHA) is a rare malignant tumor. We explored the demographic features and prognostic factors of PHA. Methods We used the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database to extract patients diagnosed with PHA from 1975 to 2016. We used the Kaplan-Meier method and Cox proportional hazards regression to evaluate the risk factors for overall survival (OS) and disease-specific survival (DSS). The nomograms were constructed and validated using the concordance index (C-index) and calibration plots. Results In total, 366 patients were included in this study. The disease onset was hidden, and most patients already had advanced disease when diagnosed. The prognosis of PHA was very poor, and the overall 6-month, 1-year and 2-year survival rates were 20.3%, 12.8% and 9.3%, respectively. Sex, age and surgery were all predictors of both OS and DSS in multivariate analysis. Women had better survival rates than men, and patients aged

A man in his 50s presented with pitting edema of both lower legs and abdominal distension as his chief complaint. His personal medical history and family history were unremarkable, except that he was a heavy drinker consuming 66 g of alcohol per day and a heavy smoker. Blood tests upon admission showed slight hepatic dysfunction, thrombocytopenia, jaundice, hypoalbuminemia, and decreased coagulability. Tumor marker tests showed elevated levels of CA19-9 and PIVKA-II. Contrast-enhanced computed tomography revealed enhancement of multiple masses predominantly in the right lobe of the liver in the early phase, followed by diffuse enhancement of the entire liver in the delayed phase. Hepatic arteriography demonstrated large hemangioma-like lesions corresponding to the masses revealed by computed tomography. That findings seemed to be cotton wool appearance. On magnetic resonance images, there were multiple mass-like lesions that showed homogeneous or heterogeneous low signal intensity on T1-weighted images, and clearly high signal intensity on T2-weighted images. The findings were atypical and no definite diagnosis could be made. Hepatic failure then rapidly worsened, and the patient died on hospital day 20. Autopsy led to the diagnosis of hepatic angiosarcoma.

Primary hepatic angiosarcoma (PHA) is a rare mesenchymal liver tumor, accounting for 0.1–2% of primary liver malignancies. The clinical presentations of PHA are variable, from asymptomatic to liver failure or complicated with tumor rupture. The diagnosis of PHA is difficult due to the lack of specific clinical manifestation and investigation results, which can be confused with other liver tumors resulting in late diagnosis. However, there is currently a paucity of effective therapeutic approaches. We advocate early diagnosis with radiological imaging and histopathology because most of them are diagnosed in late-stage and carry a grave prognosis. Surgical resection remains the mainstay of treatment, which can significantly prolong survival. Chemotherapy, including transarterial chemoembolization, is an option for palliative treatment. Unfortunately, molecular treatment has limited efficacy and liver transplantation is also not recommended due to high rate of recurrence. We present a case series of four patients with biopsy-proven PHA which had distinct presentations and clinical courses.

Background and Aims: Of all primary liver tumors, primary hepatic angiosarcoma (PHA) is a rare and aggressive malignant vascular tumor. The standard therapeutic care for hepatic angiosarcoma remains unclear. This study compared the survival outcomes of aggressive treatment (resection and liver transplant) and nonaggressive treatment (chemotherapy, transarterial chemoembolization [TACE], and conservative treatments) for patients with PHA and analyzed the prognostic factors influencing survival. Materials and Methods: Data of patients diagnosed as having PHA at our facility were retrospectively reviewed. The primary outcome was survival time. The secondary outcome was calculated baseline characteristics. Results: We included a total of 19 patients, who were divided into 2 treatment groups: aggressive (8 patients had undergone resection or transplants) and nonaggressive (11 patients had undergone TACE, chemotherapy, or conservative treatment). The mean survival time was 233.1 ± 189.7 days in the aggressive treatment group and 146.5 ± 115.8 days in the nonaggressive treatment group. A Kaplan-Meier plot revealed no significant difference in survival time between the 2 treatment groups (P = .3256). Conclusions: The survival time of patients receiving aggressive treatment was longer than that of those receiving nonaggressive treatment. The long term survival time in some selective cases of aggressive treatment will be achieved. Thought a difference was not significant between the groups. Because the number of patients was limited, more cases are required to confirm these findings.

PHA in the paediatric population is an extremely rare and aggressive malignant soft tissue neoplasm, with less than 50 cases published worldwide. The prognosis is dismal. If the tumour is unresectable, one treatment option is LT. In this article, the current available literature is reviewed and additionally, three cases of paediatric patients with PHA who underwent LT at Karolinska University Hospital, Sweden, are presented. Based on the literature and our own experience, there is undoubtedly possible good outcome of LT due to PHA. On the contrary, no patients have survived PHA without LT. PHA in paediatric patients should be recommended to LT in selected patients. Effect of modern adjuvant chemo and RT should be evaluated further based on international registry for such rare cases of PHA.

Tumor-to-tumor metastasis is a seldom reported phenomenon whereby a neoplasm seeds within another histologically distinct tumor, with only 84 cases documented in the literature. We hereby describe the case of a 95-year-old woman who died of widespread metastases identified as a primary hepatic angiosarcoma on autopsy, and the interesting finding of a seeding foci within a dural meningioma. Although meningiomas are the most common intracranial neoplasms to harbor such a phenomenon, this is to our knowledge the first case where an angiosarcoma was identified as the donor tumor.

Introduction. Angiosarcomas are malignant tumors of vascular endothelium that may arise from different locations. Although primary hepatic angiosarcoma accounts for only 1.8% of primary liver tumors, it is the most common malignant mesenchymal tumor of the liver. We report a case of primary hepatic angiosarcoma with melena as an unusual initial manifestation of this extremely rare tumor. Case report. Forty-four-years old patient with melena was referred to our Clinic because melena was not resolved after repeated argon plasma coagulation of bleeding lesions during esophagogastroduodenoscopy in the regional hospital. Abdominal ultrasound and multislice computed tomography (MSCT) revealed enlarged liver, with focal lesion 6 cm in diameter localized in the left lobe with multiple satellite lesions in both liver lobes, enlarged spleen and extremely dilated and long umbilical vein. Double-balloon enteroscopy and video capsule endoscopy detected the multiple bleeding vascular lesions in the small bowel. Histopathological examination and immunohistochemistry of the small bowel lesions revealed malignant mesenchymal proliferation with vascular/endothelium differentiation of neoplastic cells. The patient was diagnosed with metastatic angiosarcoma probably of hepatic origin with metastasis in the small bowel, that caused melena, and in the lumbar spine, causing back pain. Conclusion. Rare causes of melena include bleeding from primary or metastatic hemangiosarcoma localized in the gastrointestinal tract, especially small bowel.

Hepatic angiosarcoma is an uncommon primary malignancy of the liver. It carries a poor prognosis because of very aggressive nature of the tumor. Clinical presentation of hepatic angiosarcoma is variable, most common being hepatomegaly or abdominal mass. Here we report a case of primary hepatic angiosarcoma presented with spontaneous rupture and hemoperitoneum, which was managed successfully by transarterial embolization.

Introduction and importance Primary hepatic angiosarcoma (PHA) is a rare and aggressive liver malignancy of endothelial cell origin and is associated with poor outcome. Pre-operative confirmation of the diagnosis is challenging, as clinical and radiological findings are generally non-specific. Very rarely, spontaneous haemoperitoneum may occur due to the spontaneous rupture of previously undiagnosed PHA. Case presentation We describe a case of a 28-year-old male with haemoperitoneum due to the rupture of previously undiagnosed PHA. After failing to respond to the non-operative measures, the patient underwent emergency partial liver resection and recovered without any post-operative complications. Histopathological examination of the specimen confirmed the diagnosis of PHA. Two months after the operation, the patient represented with advanced metastatic disease and disseminated intravascular coagulation (DIC). The patient died one month after discharge. Clinical discussion A patient with PHA presents a diagnostic challenge due to its rare incidence and non-specific clinical findings. Spontaneous intra-abdominal haemorrhage can occur due to PHA rupture and carries a dismal prognosis. In addition to emergency haemorrhage control, complete surgical resection with clear margins is the definitive treatment to date, however, most cases of PHA are unresectable at diagnosis and recurrence is common even after complete resection. Conclusion PHA is associated with very poor outcomes, due to its rapid progression, early recurrence, and metastatic nature. The median survival is approximately 5 months. Haemoperitoneum secondary to rupture of previously undiagnosed PHA is uncommon and is a poor prognostic indicator. Complete surgical resection of the disease is challenging and there is no established treatment., Highlights • Primary hepatic angiosarcoma (PHA) is a rare soft tissue sarcoma. • PHA presents a diagnostic challenge due to its non-specific clinical findings. • Haemoperitoneum can rarely occur due to the spontaneous rupture of PHA. • Complete resection of PHA is the only curative treatment, however challenging. • PHA has a poor prognosis and there is no established treatment guideline.

Purpose Primary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma. Methods Among 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed. Results Three of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively. Conclusion The clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.

International audience; Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations (DNAJB1-PRKCA and CAMTA1-WWTR1 fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers.

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 angiosarcoma.

Health is prominent in mortal cheerfulness and financial progress across the world. Being healthy and active is also very important to the human lifestyle. Numerous major problems like liver disease, heart disease, and diabetes may decrease one's activity level. One of the major issues is liver abnormality. In India, most individuals suffer from the deficiency of the liver. Most liver diseases occur due to some human faults like consumption of heavy alcohol, consuming fast foods, usage of identical needles while injecting drugs, etc. This may lead to many complications in the future. Due to liver malfunctioning, many disorders may take place such as liver cancers, hepatic angiosarcoma, pediatric hepatoblastoma, hepatitis B, and hepatitis C, etc. Machine learning techniques have been strived on numerous liver disorder datasets with Python programming language to find out the accurate and exact analysis of disease. The ultimate goal of this investigation is to predict the best accuracy after successfully executing machine learning algorithms that have been used on various liver disease datasets.

A 71-year-old male who presented with right upper quadrant pain was found to have hepatic angiosarcoma. We briefly review the epidemiology, presentation, imaging findings, and pathological diagnosis of hepatic angiosarcoma.

Primary hepatic angiosarcoma was diagnosed in a 59-year-old woman who presented an arthralgia of limbs and dry cough for 6 weeks. Physical examination revealed digital clubbing. A Tc-MDP bone scintigraphy showed diffusely increased uptake along the cortical margins of long bones, suggesting hypertrophic osteoarthropathy.

Background/aim Epithelioid hemangio-endothelioma (EHE) of the liver is an uncommon vascular tumor with variable clinical courses ranging from stable disease to fatal outcome. EHE can mimic epithelioid angiosarcoma, which has a more aggressive behavior, especially in a small biopsy sample. EHEs are known to have the WWTR1-CAMTA1 fusion gene, and nuclear expression of CAMTA1 by immunohistochemistry (IHC) has been reported in about 90% of EHEs in multiple organs. Our study aimed to validate the diagnostic utility of CAMTA1 expression in EHEs, especially in the liver. Patients and methods IHC was performed using anti-CAMTA1 antibody in 34 tumors (24 hepatic EHEs and 10 angiosarcomas). In CAMTA1-negative EHEs, TFE3 IHC was performed. Results Of the 24 hepatic EHEs, 22 (91.6%) showed nuclear staining for CAMTA1. One of two CAMTA1-negative cases showed TFE3 positivity. The other case was negative for TFE3. Meanwhile, all 10 angiosarcoma cases had no CAMTA1 expression. Conclusion CAMTA1 is a highly sensitive and specific marker for diagnosis of hepatic EHE. It is helpful for differentiation of hepatic EHE and angiosarcoma, especially in small biopsy samples.

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.A systematic literature search was conducted using PubMed, Embase and Chinese Biomedical Literature database, to identify articles published from January 1980 to July 2017. Search terms were "hepatic angiosarcoma" and "liver angiosarcoma". Additional articles were retrieved through manual search of bibliographies of the relevant articles. Pooled individual data concerning the prognosis following various therapeutic modalities were analyzed.A total of 75 articles involving 186 patients were eligible for inclusion. The median overall survival (OS) was 8 months, with 1-, 3-, and 5-year OS rates of 36.6%, 22.3%, and 12.0%, respectively. The median OS after partial hepatectomy (n = 86), chemotherapy (n = 36), liver transplantation (n = 17), and supportive care (n = 46) were 15, 10, 5 and 1.3 months, respectively. Small tumor size (10 cm) was the only significant favorable factor for OS after partial hepatectomy (P = 0.012).Despite the dismal prognosis, partial hepatectomy could prolong the survival of hepatic angiosarcoma patients, particularly those with tumors10 cm. Chemotherapy could be an option for unresectable disease. Liver transplantation is not a recommendable option for the management of this malignancy.

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-EOB-DTPA-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.

Primary hepatic angiosarcoma is a rare tumor originating from endothelial cells in the liver and accounts for approximately 1% of all hepatic malignant tumors. It is difficult to diagnose due to the lack of specific symptoms or tumor markers. No effective treatment exists, but complete surgical resection may achieve a good outcome. Since most primary hepatic angiosarcomas are already at an advanced stage at diagnosis, few reports describe tumors smaller than 2 cm. We report a case of surgery for a 1.7-cm sized primary hepatic angiosarcoma. Further studies are required to improve the preoperative diagnosis of primary hepatic angiosarcoma.

In the current study, we report a case of a 46-year-old man who presented with sudden abdominal pain and was diagnosed with rupture of hepatic angiosarcoma (HAS). He underwent surgery, but died 13 days after the onset of the abdominal pain. Chronic exposure to carcinogens, such as thorium dioxide, arsenic, vinyl chloride, and radium, is associated with HAS. However, our patient had not been exposed to such carcinogens. He had submitted himself for annual medical checkups since he was employed. His liver was cirrhotic, and medical history data showed that he had had fatty liver for at least 10 years before HAS onset. Although liver cirrhosis may play a role in the occurrence of HAS, the connection of chronic fatty liver in the tumorigenesis remains unclear. Case reports regarding HAS with fatty liver are few. To the best of our knowledge, this is the first case of HAS occurring in a cirrhotic liver that advanced from persistent fatty stage. Given that HAS is a rare tumor, data collection is important for investigating its pathophysiology. Case presentations considering health conditions before HAS onset are limited; therefore, we present a case of HAS with annual health checkup data before disease onset.

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 (PHA) is a rare malignant tumor that occurs mainly in the elderly, with almost no specific symptoms or tumor markers. Information on the imaging characteristics of this tumor is limited due to its rarity. Therefore, it is difficult to diagnose PHA. So far, its definite diagnosis depends on histopathologic examination combined with immunohistochemical results. Patients with PHA have a poor prognosis in spite of surgical resection of this tumor. In this case report, we present a 72-year-old woman with PHA, focusing on the imaging features of this tumor, especially its enhancement pattern on contrast-enhanced ultrasound images. Contrast-enhanced ultrasound provided helpful information for diagnosis.

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 Association-European 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.

Background & aimPrimary hepatic angiosarcoma is a rare tumor with poor prognosis. The aim of this study was to generate a new angiosarcoma model to improve research on hepatic angiosarcoma.MethodsPigs sus scrofa were treated with different regimens of diethylnitrosamine (DENA). Tissues were analyzed by histology and immunohistochemistry. Serum parameters were determined. Angiosarcoma tissue was investigated for chromosomal aberrations by aCGH analysis.ResultsAnimals of almost all different treatment regimens developed a multitude of variable liver lesions. Different tumor types such as granulation tissue type, cellular-like, hyalinization necrosis-like, angiosarcoma-like, dysplastic nodule-like, hepatocellular-like, glandular structure-like, and leiomyoma-like lesions were observed. Weekly treatment with 15 mg/kg for up to 52 weeks or a single shot of 200 mg/kg DENA led to the development of hepatic angiosarcomas. aCGH analysis of angiosarcoma tissue revealed increased alterations in tumors compared to non-tumorous tissue. Most of the chromosomal alterations were found on chromosomes 6, 7, 12, and 14.ConclusionIn this preliminary study treatment of sus scrofa with weekly injections of 15 mg/kg DENA results in a new model for primary hepatic angiosarcoma. This model may help to shed light on the pathomechanisms of primary hepatic angiosarcoma and might therefore open new treatment options.

Hepatic Angiosarcoma is a rare, high grade, aggressive mesenchymal tumor. It is the most common malignant mesenchymal tumor of the liver, but is nonetheless rare, accounting for 2% of all primary liver malignancies, with a male predominance. Some hepatic Angiosarcoma were associated with exposure to anabolic steroids, radiation, thorium dioxide, arsenic, vinyl chloride and use of oral contraceptives, but most cases are now considered idiopathic. Given that hepatic angiosarcoma clinical, laboratory and radiologic presentation are non specific, radical resection of the tumor is being currently the best treatment option. Its diagnosis is difficult and often delayed, and requires histopathological examination. The prognosis is extremely poor with a high mortality rate and few therapeutic options. We report a case of a woman who presented with acute liver failure, which is a very unusual presentation of HAS, diagnosed with hepatic angiosarcoma through a liver biopsy. She had a rapidly worsening clinical course and died within four months of diagnosis.

Rationale: Hepatic angiosarcoma is a rare endothelial cell tumor that may lead to concurrent consumptive coagulopathies including disseminated intravascular coagulation (DIC). This report details a multifaceted approach to managing DIC in a patient with advanced-stage hepatic angiosarcoma, which continued to progress after a brief response to taxane-based chemotherapy. Patient concerns: A 55-year-old man with a recent history of hemorrhoids and hemarthroses presented with acute rectal bleeding. He was found to have concurrent hepatomegaly, abnormal liver function tests, anemia, thrombocytopenia, and coagulopathy. Diagnoses: DIC in the setting of hepatic angiosarcoma. Interventions: The patient's acute bleeding in the setting of DIC was controlled with a combination of antifibrinolytic agents to prevent clot breakdown, heparin products to prevent deposition of new clot, and romiplostim to increase platelet production. His angiosarcoma was treated with various combinations of chemotherapy, including taxane-based chemotherapy, doxorubicin, and pazopanib. Outcomes: The patient's DIC and acute bleeding on initial presentation improved following treatment with unfractionated heparin and low-molecular weight heparin maintenance therapy. It is unclear if the chemotherapy to treat the hepatic angiosarcoma played a significant role in the improvement of DIC. Lessons: Laboratory measurement of prothrombin fragment 1.2, a byproduct of prothrombin conversion to thrombin, proved to be a useful way to monitor this patient's DIC over time.

We report a case of 6-month-old infant presented with abdominal distension in whom an abdominal ultrasound and hepatic magnetic resonance imaging (MRI) revealed a large hepatic mass of malignant appearance.

Hepatic angiosarcoma is a rare and heterogeneous neoplasm that carries with it an especially poor prognosis. Diagnosis is challenging given that patients often present with vague, nonspecific complaints, and early multiorgan dysfunction has the potential to produce a number of unique clinical syndromes. Here, we present a case of a patient with hepatic angiosarcoma who developed cryptogenic cirrhosis, Kasabach-Merritt Syndrome (consumptive thrombocytopenia), and bone marrow fibrosis as a result of her malignant lesions. While parenchymal biopsy ultimately remains the gold standard for diagnosis, careful review of enhancement patterns on imaging may suggest malignant features in need of further investigation.

Primary hepatic angiosarcoma is a very rare malignancy with a poor prognosis. Because patients present with no specific symptoms, the cancer can grow undetected and most cases are diagnosed too late for resection. We present the case of a 78-year-old Japanese man admitted to our hospital with massive hematemesis and melena. A total gastrectomy had previously been performed on the patient to treat gastric cancer. Endoscopic injection sclerotherapy was performed to control the bleeding from varices over the anastomosis. Computed tomography revealed the presence of multiple atypical liver nodules in the enhanced image. Histological diagnosis of hepatic angiosarcoma was obtained by percutaneous ultrasound-guided liver biopsy. To our knowledge, this is the first report of a patient with hepatic angiosarcoma and acute variceal hemorrhage.

Hepatic angiosarcoma is a rare condition that has been associated with exposure to colloidal solutions of thorium dioxide, vinyl chloride, arsenic and radiation. Therapeutic guidelines have not been definitively established due to the small number of cases of this disease. The present study reviewed 28 cases of hepatic angiosarcoma from studies that had been published between January 2000 and December 2012, in addition to 6 cases diagnosed at Tri-Service General Hospital (Taipei, Taiwan). Clinical staging was based on American Joint Committee on Cancer staging system for soft tissue sarcoma (2014). With a mean follow-up of 27.5 months (range, 0.27–102 months), 18% (6/34) of the patients survived. The 1-, 3- and 5-year survival rates were 68.0±9.3, 42.1±10.2 and 32.7±9.8% for patients with stage I disease (mean follow-up, 32.7 months), whilst the 1- and 3-year survival rates were 33.3±15.7 and 22.2±13.9% for patients with stage IV disease (mean follow-up, 13.0 months). Determining an appropriate therapeutic strategy for this patient group is necessary. New studies encompassing larger patient populations are required in order to analyze and define standard prognostic parameters and to standardize a treatment approach for this extremely rare neoplasm.

Primary hepatic angiosarcoma (PHA) is rare, does not possess any characteristic tumor markers, is primarily observed in the elderly, and often presents with nonspecific symptoms, including discomfort or distension of the abdomen, weight loss and fatigue. Thus, PHA is difficult to diagnose, particularly if the patient presents no history of exposure to carcinogens, and its definitive diagnosis requires histological examination following surgery. Patients with PHA present poor long-term survival, and surgical resection of the tumor is currently the best treatment option for PHA. In the present report, the case of a 64-year-old man initially diagnosed with hydatid cyst, who was subsequently diagnosed with a giant PHA in the middle of the liver, is described. Further studies are required to investigate the diagnosis and treatment of PHA.

Angiosarcoma accounts for