Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma Who

Angiosarcoma is a rare and highly aggressive sarcoma subtype and occurs throughout the body at any age [1]. The prognosis of angiosarcoma is generally poor even in localized disease with 5-year overall survival (OS) up to only 60% with a median survival of around 3-10 months for metastatic disease [1-4]. In case of localized disease, standard treatment is surgical resection, sometimes combined with preoperative and postoperative radiation. However, negative surgical margins could be often difficult to achieve especially in the head and neck or in the presence of multifocal disease. These inoperable or metastatic diseases are treated with systemic chemotherapy [5]. Doxorubicin and weekly paclitaxel (wPac) are currently regarded as a preferred option for firstor secondline therapy with a median OS of approximately 8 to 10 months [6,7]. However due to the rarity of the disease, there are only limited efficacy data for such therapies. Meanwhile, several components involved in angiogenesis have been investigated as potential targets to treat angiosarcoma [8]. These include vascular endothelial growth factor (VEGF) and multiple VEGF receptors, which are the key regulators of angiogenesis that are overexpressed in angiosarcoma [9,10]. Bevacizumab is a recombinant human antibody against VEGF and has been tested in clinical trials both as a monotherapy and in combination with other drugs [11]. Multi-target tyrosine kinase inhibitors (sunitinib, sorafenib, and pazopanib) have also been applied, but the efficacy of such treatment was inconclusive as the numbers of angiosarcoma patients involved were low [1,12]. As consensus treatment strategy for angiosarcoma is not well established, it is necessary to better understand the clinicopathological features of angiosarcoma and correlate them with the treatment response and clinical outcomes. In this retrospective study, we reviewed the patients with