ABSTRACT

Introduction: Spleen angiosarcoma (SA) is a rare malignant neoplasm that arises from the splenic vascular endothelium, with only around 300 cases reported to date. Due to a limited number of reported cases, there is a paucity of data and a lack of understanding of its presentation, diagnosis, and management. In this study, we aim to provide a comprehensive review of SA.

Areas covered: On 27 February 2021, a literature search was done in PubMed and Embase database. The search yielded 122 articles involving 205 patients. The focus was on patient demographics, risk factors, clinical presentations, investigation results, preliminary diagnoses, therapies provided, and patient outcomes. These factors were analyzed to identify possible risk factors, diagnostic modalities, and therapeutic principles that were not mentioned before.

Expert opinion: The clinical presentation or investigation results of patients with SA are often nonspecific. Hence, they may not be sufficient to clinch the diagnosis of SA if used alone. The authors recommend a triple assessment of clinical examination, imaging findings, and pathology to diagnose SA with high accuracy. Splenectomy should be the mainstay of management, with chemotherapy and radiotherapy considered as adjuncts, especially in the presence of metastases.

摘要

简介：脾血管肉瘤（SA）是一种罕见的起源于脾血管内皮的恶性肿瘤，迄今仅报道约300例。由于报告的病例数量有限，缺乏数据，对其表现、诊断和管理缺乏了解。在这项研究中，我们旨在对 SA 进行全面审查。

涵盖的领域：2021 年 2 月 27 日，在 PubMed 和 Embase 数据库中进行了文献检索。搜索产生了 122 篇文章，涉及 205 名患者。重点是患者人口统计、风险因素、临床表现、调查结果、初步诊断、提供的治疗和患者结果。对这些因素进行了分析，以确定以前未提及的可能的风险因素、诊断方式和治疗原则。

专家意见：SA 患者的临床表现或调查结果通常是非特异性的。因此，如果单独使用它们可能不足以确定 SA 的诊断。作者建议对临床检查、影像学发现和病理学进行三重评估，以高精度诊断 SA。脾切除术应该是主要的治疗方法，化疗和放疗被认为是辅助治疗，特别是在存在转移瘤的情况下。