Shustef Elina, Kazlouskaya Viktoryia, Prieto Victor G, Ivan Doina, Aung Phyu P
Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Department of Dermatology, SUNY Downstate Medical Center, Brooklyn, New York, USA.
J Clin Pathol. 2017 Nov;70(11):917-925. doi: 10.1136/jclinpath-2017-204601. Epub 2017 Sep 15.
Cutaneous angiosarcoma (cAS) is a rare malignant neoplasm with variable clinical presentation. Although a distinct vascular tumour, cAS shares many overlapping histopathological features with other vasoformative and epithelioid tumours or 'mimickers'. cAS shows aggressive behaviour and carries a grave prognosis, thus early diagnosis is of paramount importance to achieve the best possible outcomes. Recently, several genetic studies were conducted leading to the identification of novel molecular targets in the treatment of cAS. Herein, we present a comprehensive review of cAS with discussion of its clinical, histopathological and molecular aspects, the differential diagnosis, as well as current therapies including ongoing clinical trials.
皮肤血管肉瘤(cAS)是一种临床表现多样的罕见恶性肿瘤。尽管cAS是一种独特的血管肿瘤,但它与其他血管形成性和上皮样肿瘤或“模仿者”有许多重叠的组织病理学特征。cAS表现出侵袭性,预后严重,因此早期诊断对于取得最佳治疗效果至关重要。最近,开展了几项基因研究,从而确定了cAS治疗中的新分子靶点。在此,我们对cAS进行全面综述,讨论其临床、组织病理学和分子方面、鉴别诊断以及包括正在进行的临床试验在内的当前治疗方法。