Angiosarcoma is a very rare soft tissue sarcoma that originates from endothelial cells and typically has a poor prognosis. It is most commonly found in elderly white men and can occur anywhere in the body, particularly in the head, neck, and scalp. Patients who have undergone previous radiation treatment or who have chronic lymphedema also face an elevated risk of this condition. Various genetic changes are suspected to contribute to the development of angiosarcoma, and these changes have been identified as potential targets for treatment. For localized disease, wide surgical resection is often the prudent course of action. A multidisciplinary approach, which may include surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes. In this review, we discuss the general understanding of angiosarcoma and its management, with a particular focus on the current evolving treatments for the disease.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10622948 | PMC |
| http://dx.doi.org/10.7181/acfs.2023.00409 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Novel Therapeutics in Soft Tissue Sarcoma.
Cancers (Basel)
December 2024
Sarcoma Unit, The Royal Marsden Hospital and Institute of Cancer Research, London SW3 6JZ, UK.
There has been noteworthy progress in molecular characterisation and therapeutics in soft tissue sarcomas. Novel agents have gained regulatory approval by the FDA. Examples are the tyrosine kinase inhibitors avapritinib and ripretinib in gastrointestinal stromal tumours (GIST), the immune check point inhibitor atezolizumab in alveolar soft part tissue sarcoma, the γ-secretase inhibitor nirogacestat in desmoid tumours, the NTRK inhibitors larotrectinib and entrectinib in tumours with fusions, the mTOR inhibitor nab-sirolimus in PEComa, and the EZH-2 inhibitor tazemetostat in epithelioid sarcoma.
View Article and Find Full Text PDFHistologic and immunologic factors associated with response to immune checkpoint inhibitors in advanced sarcoma.
Clin Cancer Res
December 2024
Stanford University, Palo Alto, California, United States.
Purpose: To characterize factors associated with response to immune checkpoint inhibitors (ICIs) in advanced sarcoma.
Experimental Design: This is a retrospective study with a cohort of 216 patients with advanced sarcoma treated with ICIs between 2016-2023 at Stanford Health Care. Overall survival (OS), progression free survival (PFS), objective response rates per RECIST criteria (ORR), and reason for ICI discontinuation were analyzed across histologic subtypes, ICI regimens, tumor mutational burden (TMB), and PD-L1 expression.
Count Me In: patient-partnered research to address disparities for rare cancer patients.
Ther Adv Rare Dis
December 2024
Count Me In, Cambridge, MA, USA.
Background: Approximately 25% of cancer patients are diagnosed with rare cancers and face unique challenges. Decentralized patient-partnered research efforts, like Count Me In provide an avenue for patients to participate in research that overcomes key barriers to address disparities in rare cancer research to accelerate discovery.
Objectives: Projects in metastatic breast cancer (The Metastatic Breast Cancer Project; MBCproject) and angiosarcoma (The Angiosarcoma Project; ASCproject) highlight disparities that exist for all cancer patients and underscore those that are compounded for rare cancer patients.
Demographics, Prognostic Factors, and Survival Outcomes in Hepatic Angiosarcoma: A Retrospective Analysis.
J Gastrointest Cancer
December 2024
Department of Pathology, Texas Tech University Health Sciences Center, 3601 4Th ST, Lubbock, TX, USA.
Article Synopsis
- Hepatic angiosarcoma (HA) is a rare cancer with no standardized treatment protocols; a study aims to analyze clinicopathologic data, prognostic factors, and treatment outcomes.
- From 2000 to 2021, data from 389 HA patients showed a low overall 3-year survival rate of only 6.7%, with surgical resection offering the best outcome at a 1-year survival rate of 18.20%.
- Key negative prognostic factors included age over 70, male sex, and distant tumor stage, while female sex appeared to be a positive factor, suggesting a need for improved management strategies.
Investigating intra-tumoural heterogeneity and microenvironment diversity in primary cardiac angiosarcoma through single-cell RNA sequencing.
Clin Transl Med
December 2024
Department of Cardiovascular Surgery, School of Medicine, the First Affiliated Hospital of Zhejiang University, Hangzhou, China.
Background: Primary cardiac angiosarcoma (PCAS) is a rare and aggressive heart tumour with limited treatment options and a poor prognosis. Understanding cellular heterogeneity and tumour microenvironment (TME) is crucial for the development of effective therapies. Here, we investigated the intratumoural heterogeneity and TME diversity of PCAS using single-cell RNA sequencing (scRNA-seq).
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!