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This study aims to provide a comprehensive understanding of primary hepatic angiosarcoma, a rare and aggressive malignancy, focusing on its diagnostic challenges and unique imaging characteristics. The objective is to delineate the distinctive features of angiosarcoma through computed tomography and magnetic resonance imaging modalities, contributing to improved diagnostic precision and adding valuable insights to the scientific literature. We present the case of a 25-year-old male with primary hepatic angiosarcoma, emphasizing the challenges in distinguishing it from other vascular tumors. [ABSTRACT FROM AUTHOR]

Background: Enhanced imaging techniques have the overwhelming advantages of being noninvasive and sensitive enough to evaluate the microcirculation of lesions, thus making them accurate in the diagnosis of hepatic lesions. Unfortunately, there is very little research on and knowledge of the imaging features of a rare cancerous condition: hepatic angiosarcoma (HA). Case summary: In this study, we retrospectively collected the data of six patients who underwent both contrast-enhanced ultrasound (CEUS) and contrast-enhanced computed tomography (CECT), and subsequently obtained a definitive histopathologic diagnosis of HA. We described the imaging appearances of HA by comparing CEUS and CECT images. Furthermore, we analyzed these imaging characteristics from the perspective of histopathology and tumorigenesis. The study included the largest number (six) of histopathologically confirmed HA patients who had received CEUS examinations to date. Conclusion: By offering readers comprehensive knowledge of contrast imaging, especially CEUS, in the diagnosis of HA, our study may reduce misdiagnosis and further improve treatment options. [ABSTRACT FROM AUTHOR]

An 80-year-old Japanese man presented to our hospital with intra-abdominal hemorrhage due to a ruptured liver tumor. Transcatheter arterial embolization (TAE) temporarily achieved hemostasis, but he died following re-rupture 4 days later. Based on autopsy findings, the liver tumor was diagnosed as hepatic angiosarcoma. Embolic agents used during embolization were identified within the hepatic small interlobular arteries. However, there were no findings of tumor cell necrosis or ischemic change in the angiosarcoma. In the present case, TAE alone did not induce ischemia-induced tumor necrosis, suggesting that TAE might be unsuitable to treat hepatic angiosarcoma. Treatment optimization for ruptured hepatic angiosarcoma is desired.

An 80-year-old Japanese man presented to our hospital with intra-abdominal hemorrhage due to a ruptured liver tumor. Transcatheter arterial embolization (TAE) temporarily achieved hemostasis, but he died following re-rupture 4 days later. Based on autopsy findings, the liver tumor was diagnosed as hepatic angiosarcoma. Embolic agents used during embolization were identified within the hepatic small interlobular arteries. However, there were no findings of tumor cell necrosis or ischemic change in the angiosarcoma. In the present case, TAE alone did not induce ischemia-induced tumor necrosis, suggesting that TAE might be unsuitable to treat hepatic angiosarcoma. Treatment optimization for ruptured hepatic angiosarcoma is desired. [ABSTRACT FROM AUTHOR]

Primary hepatic angiosarcoma (PHA), a rare soft tissue tumor, accounts for only 2% of all liver malignancies. Pathologically challenging, PHA is difficult to be distinguished from other malignancies with ultrasound, Computed Tomography (CT), or Magnetic Resonance Imaging (MRI). Due to late diagnosis and resistance against traditional chemotherapy and/or radiotherapy, only 3% of PHA patients can survive up to two years after diagnosis. To our best knowledge, this case report presents the first case of an advanced diffuse PHA with ruptured hemorrhage that has been effectively treated with TACE and Anlotinib plus Camrelizumab. So far, the patient has received 10 cycles of treatment and is faring well. Latest MRI results show that the tumor has shrunk by 56% and can be assessed as a partial response (PR). This case report includes our experience in treating such a advanced malignancy, and we hope that larger studies on advanced PHA can better quantify the potential benefit. [ABSTRACT FROM AUTHOR]

Primary splenic or hepatic angiosarcomas are ultra-rare and aggressive malignancies associated with poor prognosis. The mainstay treatments are surgical resection and chemotherapy. We report a case of angiosarcoma in a 50-year-old woman who presented with bruising, fatigue, ecchymosis, and hepatosplenomegaly. She was treated with the multi-kinase inhibitor sunitinib for 4 weeks before developing a splenic hemorrhage and succumbing. Recent studies have demonstrated the clinical benefit of immunotherapies in angiosarcomas. Additionally, sequencing techniques have showcased the diverse molecular aberrations involved in angiosarcomas, which offer opportunities for precision-matched targeted therapies such as inhibitors of the VEGF/VEGFR axis and PI3K/Akt/mTor pathway.

Hepatic angiosarcoma is rare and accounts for 2% of all primary liver malignancies. Most patients die within 6 months from the time of diagnosis. Surgical resection is the only radical treatment, with a median survival time of around 1.5 years. However, the role of surgery for ruptured tumors has not been determined. A 49-year-old gentleman treated of ruptured hepatic angiosarcoma treated with surgical resection who survived for 11 months is reported here. [ABSTRACT FROM AUTHOR]

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.

Hepatic angiosarcoma is a rare and aggressive liver tumor. We report a case study of an 82-year-old elderly gentleman who presented with failure to thrive and ascites. Initially suspected to be cirrhosis, biopsy results eventually concluded angiosarcoma of the liver. Our patient presented with an infiltrative form, rather than distinct masses, which led to portal hypertension and ascites. The variance in symptomatology and radiology presentations make a diagnosis of hepatic angiosarcoma challenging and require a high index of suspicion., (© 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)

A hepatic hemangioma is a benign liver tumor made up of a number of blood-filled chambers surrounded by liver-supplied endothelial cells. Most liver hemangiomas are asymptomatic and are only discovered during imaging studies for other conditions. Ultrasound is used for initial screening followed by a computed tomography scan, which shows slow enhancement due to small vessels and can be used to diagnose the location, number, and size of a hepatic hemangioma. A large liver hemangioma can range in size from 10 centimeters to more than 20 centimeters and can cause symptoms and complications that require prompt intervention. Hepatic hemangiomas can co-occur with other localized hepatic lesions; a careful diagnosis is necessary to distinguish them. In this case study, a 48-year-old woman complained of a stomachache that had persisted for three months. Following an initial clinical evaluation, hepatomegaly was found, and contrast-enhanced computed tomography (CECT) abdomen and pelvis was performed, revealing numerous giant hepatic hemangiomas. Significant improvements were noted in the patient's condition with tumor embolization., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Gopalan et al.)

Primary hepatic angiosarcoma (PHA) is a rare and aggressive mesenchymal liver tumor with a poor prognosis and high mortality. Treatment options are limited to palliative chemotherapy with surgical resection reserved for the few cases that present early. We present a case of a patient who presented with jaundice and elevated liver enzymes. Imaging identified a diffusely heterogeneous liver consistent with cirrhosis, findings of portal hypertension, and 2 ill-defined liver lesions. Biopsy results confirmed PHA. Primary hepatic angiosarcoma does not have a typical presentation but should be considered for any patient presenting with an infiltrative liver mass. [ABSTRACT FROM AUTHOR]

Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of the liver, and data on patient outcome after surgical treatment are scarce. The aim of this study was to evaluate postoperative morbidity and overall survival (OS) of patients who underwent hepatectomy for PHA. This is a bicentric retrospective analysis of all consecutive patients who underwent liver resection in curative intent for PHA between 2012 and 2019 at the University Hospital of Muenster and the University Hospital of Bern. Nine patients (five female, four male) were included from both centers. Median age was 72 years (44–82). Most lesions (77.8%) were larger than 5 cm, and mean size of the biggest lesion was 9.4 ± 4.5 cm. Major hepatectomy was performed in four (44.4%), and radical resection (R0) was achieved in six (66.7%) patients. Postoperative complication rate was 88.8%, including 44.4% higher than 3a in the Clavien–Dindo classification. OS survival rates at 1, 2, and 3 years were 44.4%, 22.2%, and 12.5%, respectively, and median OS was 5 months. OS was significantly better after radical resection (R0: 15 months vs. R1: 0 months, p = 0.04), whereas presentation with tumor rupture at diagnosis was associated with the worst OS (0 months vs. 15 months, p = 0.005). Disease recurrence occurred in three patients (33.3%) between three and seven months after surgery. Radical resection remains the only potentially curative treatment option for PHA. However, postoperative morbidity is high, and the overall prognosis remains poor. Multimodal therapy options and management strategies are urgently needed and could improve the prognosis of patients suffering from PHA in the future. [ABSTRACT FROM AUTHOR]

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.

A 67-year-old man presented with hemorrhagic shock due to the rupture of hepatic tumor and underwent emergency partial resection of the right liver. Pathological examination revealed hepatic angiosarcoma with involvement in its surgical margin. Six months after the operation, disease recurrence was detected, and he was referred to our hospital for second opinion. CT revealed tumors at the liver cut surface and left lateral segment. The tumor at the liver cut surface abutted to the common bile duct and the portal vein. The tumor was deemed unresectable, and systemic chemotherapy with 4 courses of weekly paclitaxel was given with excellent response. Then, we performed partial liver resection of S4 and S1 with remnant right liver and middle hepatic vein, and wedge resection for the metastatic lesion of segment 3 as a conversion surgery. He developed a grade B bile leakage postoperatively and was discharged on postoperative day 28. He remained disease free for 8 months after the operation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

Abstract Background Hepatic angiosarcoma is a rare malignant tumor featured by highly aggressive behavior and poor prognosis. There are few reports about diffused hepatic angiosarcoma with Kasabach-Merritt syndrome till now. Case presentation A male patient with the chief complain of hepatic space-occupying lesion accompanied by disturbance of consciousness and jaundice. Hyperbilirubinemia, anemia, thrombocytopenia, prolonged prothrombin time, hypofibrinogenemia, decreased prothrombin activity, and increased fibrinogen degradation product and D-dimer were confirmed by blood analysis; multiple focal hypodense lesions in liver was detected by abdominal computed tomography. Liver failure and Kasabach-Merritt syndrome induced by hepatic hemangioma was diagnosed before operation and liver transplantation was performed. Hepatic angiosarcoma was finally proven by postoperative pathology. This patient died of tumor metastasis 2 months after operation. Conclusions Hepatic angiosarcoma which can generate Kasabach-Merritt syndrome and even liver failure has an extremely poor prognosis; liver transplantation option should not be considered in hepatic angiosarcoma regardless of the reason.

Background. Hepatic angiosarcoma is an uncommon, malignant, primary liver tumor, comprising 2% of liver cancers and accounting for < 1% of all sarcomas. Patients usually present with nonspecific symptoms, such as fatigue, weight loss, right upper quadrant pain, anemia, which leads to late diagnosis of an advanced stage tumor. The median life expectancy after the diagnosis of hepatic angiosarcoma is about 6 months, with only 3% of patients surviving more than 2 years. Liver failure and hemoperitoneum are the leading causes of death in patients with liver angiosarcoma. In rarer cases, it might cause paraneoplastic syndromes such as disseminated intravascular coagulopathy. The treatment of angiosarcomas is complicated as there are no established and effective treatment guidelines due to the tumor’s low frequency and aggressive nature. Case summary. We present the case of a 68-year old woman who was admitted to the hospital due to fatigue and severe anemia (hemoglobin 65 g/l). Laboratory results also revealed high-grade thrombocytopenia (8 × 109/l). The abdominal ultrasound and computed tomography scan showed multiple lesions throughout with hepatic angiosarcoma. The treatment with first-line chemotherapy (doxorubicin) was initiated despite ongoing paraneoplastic syndrome – disseminative intravascular coagulopathy. However, the disease was terminal, and the patient died 2 months since diagnosed. Conclusions. Hepatic angiosarcoma is a rare and terminal tumor. Therefore, knowledge about its manifestations and effective treatment methods is lacking. Disseminative intravascular coagulopathy is a unique clinical characteristic of angiosarcoma seen in a subset of patients.

Summary: 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.

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.

Angiosarcomas (AS) are a diverse group of soft tissue sarcomas, arising from blood and lymphatic vessels. They frequently present in the elderly, and in patients with previous radiation or lymphedema. A wide range of genetic derangements contribute to their development, and AS histology is often high-grade in keeping with aggressive disease biology. The clinical presentation, while often innocuous, is marked by its infiltrative and aggressive nature, with a proclivity for metastatic spread, and outcomes are often poor. Surgery is performed for localized, resectable cases. A multidisciplinary approach, appropriately employing surgery, radiation, chemotherapy, or potentially recently approved immune-oncology agents, can result in positive outcomes. [ABSTRACT FROM AUTHOR]

Background: Hepatic angiosarcoma is a rare malignant tumor featured by highly aggressive behavior and poor prognosis. There are few reports about diffused hepatic angiosarcoma with Kasabach-Merritt syndrome till now.Case Presentation: A male patient with the chief complain of hepatic space-occupying lesion accompanied by disturbance of consciousness and jaundice. Hyperbilirubinemia, anemia, thrombocytopenia, prolonged prothrombin time, hypofibrinogenemia, decreased prothrombin activity, and increased fibrinogen degradation product and D-dimer were confirmed by blood analysis; multiple focal hypodense lesions in liver was detected by abdominal computed tomography. Liver failure and Kasabach-Merritt syndrome induced by hepatic hemangioma was diagnosed before operation and liver transplantation was performed. Hepatic angiosarcoma was finally proven by postoperative pathology. This patient died of tumor metastasis 2 months after operation.Conclusions: Hepatic angiosarcoma which can generate Kasabach-Merritt syndrome and even liver failure has an extremely poor prognosis; liver transplantation option should not be considered in hepatic angiosarcoma regardless of the reason. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 an extremely rare diagnosis in children, with fewer than 50 pediatric cases reported in the literature worldwide. This aggressive vascular sarcoma carries a very dismal prognosis and is known to be resistant to radiation, chemotherapy, and other vascular‐targeted agents. Complete surgical resection is felt to provide the best chance for long‐term survival. In patients with tumors not amenable to resection, a liver transplant can be considered. However, very few such transplants have been reported, given that they remain controversial due to high cancer recurrence and mortality post‐transplant. Herein, we report the unique case of a 2‐year‐old child with localized hepatic angiosarcoma not amendable to resection who successfully underwent a liver transplant and received chemotherapy with six cycles of doxorubicin, docetaxel, and ifosfamide. [ABSTRACT FROM AUTHOR]

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.

The article presents a case study of six-year-old male child who is a long-time survivor of pediatric hepatic angiosarcoma (PHA) without metastasis including growth disturbance and cardiac toxicity. She develops abdominal pain and a mass in the right upper quadrant. It recommends aggressive surgical resection with postoperative adjuvant therapy including doxorubicin, pirarubicin and ifosfamide.

Mesenchymal neoplasms of the liver can be diagnostically challenging, particularly on core needle biopsies. Here, I discuss recent updates in neoplasms that are specific to the liver (mesenchymal hamartoma, undifferentiated embryonal sarcoma, calcifying nested stromal-epithelial tumor), vascular tumors of the liver (anastomosing hemangioma, hepatic small vessel neoplasm, epithelioid hemangioendothelioma, angiosarcoma), and other tumor types that can occur primarily in the liver (PEComa/angiomyolipoma, inflammatory pseudotumor-like follicular dendritic cell sarcoma, EBV-associated smooth muscle tumor, inflammatory myofibroblastic tumor, malignant rhabdoid tumor). Lastly, I discuss metastatic sarcomas to the liver, as well as pitfalls presented by metastatic melanoma and sarcomatoid carcinoma., (Copyright © 2023 Elsevier Inc. All rights reserved.)