血管肉瘤(AS)是一种罕见的侵袭性血管肉瘤,与其他肉瘤相比,在临床管理方面存在明显的挑战。虽然目前的欧洲医学肿瘤学会(ESMO)肉瘤治疗临床实践指南适用于AS,其独特的侵袭性和多样化的肿瘤表现需要专门和详细的临床建议,目前缺乏的。值得注意的是,关于手术范围的考虑,放射治疗(RT),新辅助/辅助化疗在局部疾病中差异显著,取决于每个不同的发病部位。的确,AS是对细胞毒性化疗最敏感的肉瘤类型之一。尽管如此,关于不同临床表现的最佳管理的不确定性仍然存在,强调需要通过临床试验进行进一步研究。意大利肉瘤组织(ISG)于4月1日组织了一次共识会议,2023年,在CastelSanPietro,意大利,汇集了来自多个学科的意大利肉瘤专家和“SofianelCuoreOnlus”和ISG患者倡导工作组的患者代表。目的是在现有的肉瘤临床实践指南框架内,为管理局部AS制定具体的临床建议。考虑ISG机构之间潜在的实践差异。目的是尝试规范和协调临床实践,或者至少强调当地疾病管理中的悬而未决的问题,为本地化AS的最佳方法定义最佳的循证实践,并生成本文提出的建议。
Angiosarcoma (AS) represents a rare and aggressive vascular sarcoma, posing distinct challenges in clinical management compared to other sarcomas. While the current European Society of Medical Oncology (ESMO) clinical practice
guidelines for sarcoma treatment are applicable to AS, its unique aggressiveness and diverse tumor presentations necessitate dedicated and detailed clinical recommendations, which are currently lacking. Notably, considerations regarding surgical extent, radiation therapy (RT), and neoadjuvant/adjuvant chemotherapy vary significantly in localized disease, depending on each different site of onset. Indeed, AS are one of the sarcoma types most sensitive to cytotoxic chemotherapy. Despite this, uncertainties persist regarding optimal management across different clinical presentations, highlighting the need for further investigation through clinical trials. The Italian Sarcoma Group (ISG) organized a
consensus meeting on April 1st, 2023, in Castel San Pietro, Italy, bringing together Italian sarcoma experts from several disciplines and patient representatives from \"Sofia nel Cuore Onlus\" and the ISG patient advocacy working group. The objective was to develop specific clinical recommendations for managing localized AS within the existing framework of sarcoma clinical practice
guidelines, accounting for potential practice variations among ISG institutions. The aim was to try to standardize and harmonize clinical practices, or at least highlight the open questions in the local management of the disease, to define the best evidence-based practice for the optimal approach of localized AS and generate the recommendations presented herein.