BACKGROUND: While available systemic treatments have modest long term efficacy in advanced angiosarcoma, immunotherapy represents an interesting new therapeutic opportunity. To establish its benefit, it is required to conduct a clinical trial assessing its efficacy and toxicity compared to standard treatments.
METHODS: This is a literature review from PubMed search.
RESULTS: Several systemic treatments (chemotherapy and TKI) are currently used in advanced angiosarcoma with ORR ranging from 12.5 to 68 % and PFS from 2 to 7 months. However, few randomized trials, mainly phase II, has been conducted to compare these treatments. While most centers propose doxorubicin containing regimens or paclitaxel in 1st or 2nd line, a high heterogeneity of regimens administered in this setting is observed even across sarcoma specialized centers with no consensual standard treatment. Encouraging signals of immunotherapy activity have been reported in angiosarcoma from several retrospective and phase II studies assessing anti-PD1 either alone or in combination with anti CTLA4 or TKI. Although cutaneous and head and neck location seems to benefit more from immunotherapy, response may be observed in any angiosarcoma subtype. In sarcoma in general and AS in particular, no biomarker has been clearly established to predict the efficacy of immunotherapy: high tumor mutational burden and presence of tertiary lymphoid structures are under assessment.
CONCLUSIONS: Even essential, developing a randomized clinical trial in AS struggles with the heterogeneity of the disease, the lack of consensual standard regimen, the uncertainty on optimal immunotherapy administration and the absence of established predictive biomarkers.
CONCLUSIONS: International collaboration is essential to run randomized trial in advanced AS and asses the efficacy of immune therapy in this rare and heterogeneous disease.

Primary Breast Angiosarcoma (PBA) is an exceptionally rare form of breast cancer, accounting for less than 0.05% of all breast cancers. It is characterized by a high level of malignancy, invasiveness, and has a prognosis that is typically poor. The lack of distinctive clinical features makes it prone to underdiagnosis and misdiagnosis. This study retrospectively examines a case utilizing multimodal ultrasound imaging techniques (including 2D ultrasound, contrast-enhanced ultrasound, and ultrasound elastography) for diagnosing PBA. Furthermore, the study reviews relevant literature to summarize the ultrasound characteristics of PBA, with the aim of improving understanding of this elusive condition.

Angiosarcomas originating from the gastrointestinal tract are rare but highly aggressive tumors with poor prognosis. These tumors can be misdiagnosed as benign and malignant gastrointestinal tract lesions. The definitive histological diagnosis of angiosarcomasis made by pathologists based on immunohistochemical analysis demonstrating cluster of differentiation 31 (CD31), factor VIII-related antigen (FVIIIRAg), erythroblast transformation specific related gene (ERG), and cluster of differentiation 34 (CD34). Angiosarcomas are treated with a single or multimodality approach that may include resection, radiotherapy, chemotherapy, and palliative care, depending on the stage of disease and the condition of the patient. No matter the treatment option, metastasis and death rates are substantially highin patients with angiosarcoma. In this context, a 59-year-old male with synchronous double primary angiosarcoma arising from the gastric and rectum who presented with the complaint of abdominal pain and distention to the outpatient clinic is presented in this case report, along with a brief literature review.

BACKGROUND: Cutaneous angiosarcoma (cAS) is a highly aggressive malignancy arising from the vascular endothelium. Given its rarity, there is insufficient data detailing patient demographics, management, and survival outcomes.
OBJECTIVE: To systematically compile published patient-level cases of cAS and to quantify and analyze data on demographics, management, and outcomes while determining prognostic indicators.
METHODS: Searches of EBSCOhost, MEDLINE, EMBASE, and the Cochrane Library generated 1,500 cases of cAS with individual level data available. PRISMA guidelines were followed.
RESULTS: Cutaneous angiosarcoma presented most often on the scalp of elderly men. Metastasis occurred in 36.3% of cases. Aggregate 5-year survival was 31.6% with the median survival of 25 months. The best 5-year survival was in the radiation-associated subtype (48.8%), whereas the worst was in the Stewart-Treves subtype (21.6%). Using multivariate analysis, gender, age group, disease subtype, treatment modality, and metastasis at presentation had significant effects on survival outcomes ( p < .05).
CONCLUSIONS: The breadth of information obtained enables this study to serve as a resource that clinicians may reference when they encounter cAS.

BACKGROUND: Angiosarcoma of the gallbladder is a rare diagnostic entity rarely encountered by pathologists and has rarely been reported in literature. This review aimed to examine the clinicopathological features, immunohistochemistry, treatment, and outcomes of gallbladder angiosarcoma.
METHODS: A search of the PubMed, Science Direct and Google Scholar was done with the search terms (\"angiosarcoma\" OR \"angiosarcomas\") AND (\"gallbladder\" OR \"gallbladders\"). Based on inclusion and exclusion criteria, only case reports could be used for this review.
RESULTS: 8 case reports were chosen in the end for analysis. The mean age of the patients at presentation was 65 years. It was most frequently observed in males. Abdominal pain and palpable mass were the most commonly reported symptoms. Cholelithiasis and anemia were also reported. On histopathology morphologically epithelioid appearance of angiosarcoma was evident. Cytokeratin (CK) AE1/AE3, Von willebrand factor, Factor VIII antigen, Vimentin, CD31 were positive. Meanwhile, UEA, CD34, CD117, S-100, Keratin, EMA, and CEA showed negative outcome. Surgery was the preferred method of treatment and a mean 10-months follow-up was done.
CONCLUSIONS: Despite the unavailability of convincing data, histological and immunohistochemical analyses play a major role in the diagnosis of gallbladder angiosarcoma. Nevertheless, more comprehensive clinical studies are required to provide universal guidelines for the treatment and diagnosis of angiosarcoma of the gallbladder.

UNASSIGNED: Angiosarcomas are malignant neoplasms that originate from endothelial cells. The symptoms exhibit a non-specific nature, and achieving a preoperative diagnosis is frequently challenging. They are seldom encountered in the abdomen, and their occurrence in the pancreas is even rarer.
UNASSIGNED: Here we document a 67-year-old man with pancreatic angiosarcoma and analyse the literature to outline the clinicopathologic characteristics of this rare phenomenon.
UNASSIGNED: This patient with family history of pancreas cancer presented with abdominal pain, and the CT-scan revealed a 4 cm mass at the neck of the pancreas but CA19-9 was normal. Radiologic findings were unusual for ordinary pancreas cancer. Fine-needle aspiration biopsy through endoscopic ultrasound revealed \"undifferentiated malignant cells for which the diagnosis of \"carcinoma\" was favoured. Total pancreatectomy, splenectomy and portal vein reconstruction were performed and epithelioid angiosarcoma were diagnosed. Despite an uneventful postoperative period, discharge on postoperative day 8 without any complications, as well as diligent post-discharge clinical care, the patient died 65 days postoperatively, attributed to the presence of extensive metastasis. A comprehensive literature search has identified a limited number of documented cases of primary pancreatic angiosarcoma, with only ten cases reported to date.
UNASSIGNED: Pancreatic angiosarcomas are very rare and prone to misdiagnosis. The formation of a more demarcated but high-grade tumour with necrosis is a feature that distinguishes angiosarcomas from ordinary carcinomas of this organ. Pathologic diagnosis is also highly challenging closely resembling undifferentiated carcinomas. Angiosarcomas are highly aggressive when they occur in the pancreas. Prompt diagnosis at an early stage is crucial as surgery with curative intent serves as the primary treatment approach.
Surgery with curative intent is the mainstay treatment for pancreatic angiosarcoma when diagnosed at an early stage.Oncological treatment options should be taken into consideration according to the follow-up data.Why does this paper matter?This article is important in that it is the most comprehensive review of the literature on pancreatic angiosarcoma, which is a very rare pathology, from the perspective of radiology, pathology and surgery.

Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications. Nevertheless, primary vascular bone tumors still represent a challenge for medical oncologists. Given their rarity and heterogeneity, in the last few years, there has been no significant progress in medical treatment options, so further research is needed. Here we present a review of the current knowledge regarding primary vascular tumors of the bone, correlating clinicopathologic features with tumor behavior and therapeutic approaches.

BACKGROUND: Primary cardiac angiosarcomas (PCA) is a rare malignancy with a poor prognosis. Currently, there is no standard treatment protocol for the PCA. We report a case of PCA in a 51-year-old woman.
METHODS: A 51-year-old woman initially presented with unexplained palpitations and chest tightness accompanied by nausea and vomiting, which worsened after activity and improved after rest. After symptomatic treatment, the symptoms improved, and the above symptoms recurred 8 months later.
METHODS: Positron emission tomography-computed tomography revealed multiple lung nodules of varying sizes, some of which exhibited increased glucose metabolism. Furthermore, a soft tissue mass protruding into the pericardial cavity and involving the adjacent right atrium was observed in the right pericardium. The mass exhibited increased glucose metabolism, suggestive of a pericardial tumor with multiple lung metastases. Finally, histopathologic diagnosis of metastatic angiosarcoma was done by computed tomography-guided percutaneous lung and mediastinal biopsy.
METHODS: The patient was treated with palliative chemotherapy for the primary cardiac angiosarcomas and hematogenous lung metastasis. One cycle later, the result of Next-Generation Sequencing showed that the microsatellite instability status was determined to be low-level. Based on this result, tislelizumab was added to the original chemotherapy regimen.
RESULTS: Unfortunately, the patient with PCA passed away after only 2 cycles of chemotherapy, and the cause of death remained unknown.
CONCLUSIONS: This case report well demonstrates typical imaging findings of a rare cardiac angiosarcomas and emphasizes importance of early investigation for accurate diagnosis and proper management of the cardiac angiosarcomas.

BACKGROUND: Paranasal sinus angiosarcoma is an uncommon malignancy, with only a few reported cases worldwide. Although it exhibits multiple symptoms, facial paralysis has not been previously documented as a noticeable presentation.
METHODS: In this case, we report a 40-year-old male who presented with facial numbness and pain for one month, weakness of his facial muscles for 15 days, and recurrent right epistaxis for 1 year. He had a history of nasal inflammatory polyps with chronic sinusitis. Computed tomography and magnetic resonance imaging showed space-occupying lesions in the right nasal cavity and maxillary sinus, with bone destruction occurring in the sinus wall and turbinate. This patient then underwent endoscopic surgery. According to the histopathological and immunohistochemical results, he was eventually diagnosed with paranasal sinus angiosarcoma in April 2021. To date, this patient has not initiated any radiotherapy or chemotherapy and has survived with lymphatic metastasis for at least 3 years.
CONCLUSIONS: This manuscript suggests that paranasal sinus angiosarcoma can present with facial paralysis. Moreover, pathological and immunohistochemical tests are still vital for diagnosing paranasal sinus angiosarcoma and differential diagnosis. Additionally, regular follow-up is crucial for patients with paranasal sinus angiosarcoma, enabling monitoring of recurrence, metastasis, and recovery while contributing valuable clinical data to understanding this rare disease and associated research endeavours.

Angiosarcoma is an uncommon malignant mesenchymal neoplasm, accounting for 1-2% of all sarcomas. More than half are cutaneous, with the remainder arising in the deep soft tissue, breast, bone or viscera, particularly the liver, spleen and heart. Mediastinal angiosarcomas are exceedingly uncommon. While epithelioid morphology is sometimes a minor component in conventional angiosarcoma, tumors with a predominance of epithelioid morphologic features are designated as epithelioid angiosarcoma (EAS). This is a report of a 58-year-old woman presenting with severe chest pain, accompanied by worsening dyspnea and dysphagia. Chest computed tomography (CT) revealed a large pericardial effusion and a bulky mediastinal mass. Biopsy revealed a malignant neoplasm with vascular differentiation consistent with high-grade EAS. By immunohistochemistry, epithelioid angiosarcomas express endothelial cell markers, such as CD31, CD34, ERG and FLI-1. A variable proportion express low molecular weight cytokeratin (CK), epithelial membrane antigen (EMA) and CD30. The use of molecular techniques has proven useful in the diagnosis of this rare neoplasm. Targeted next generation sequencing showed aberrations in multiple genes including NRAS, KRAS, MYC and TP53.