Clinical, dermoscopic and histopathological features of spontaneous scalp or face and radiotherapy-induced angiosarcoma

Clinical, dermoscopic and histopathological features of spontaneous scalp or face and radiotherapy-induced angiosarcoma
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DOI:
10.1111/j.1440-0960.2012.00943.x
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发表时间:
2013-08-01
影响因子:
2
通讯作者:
Piana, Simonetta
Piana, Simonetta
中科院分区:
医学4区
文献类型:
--
作者:
Zalaudek, Iris;Gomez-Moyano, Elisabeth;Piana, Simonetta

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皮肤血管肉瘤是一种罕见的恶性血管肿瘤,预后差。1,2目前已知的主要有四种类型,即:头皮和面部的散发性血管肉瘤(AS)、水肿相关性血管肉瘤(LAS)、放射诱导性血管肉瘤(RIA)和最近描述的罕见侵袭性上皮样亚型。3,4在这四种变异中,头皮和面部的血管肉瘤,也称为Wilson-Jones血管肉瘤、老年性血管肉瘤或恶性血管内皮瘤是最常见的。该疾病主要影响老年人,男性占优势。LAS在乳房切除术或其他肿瘤或手术干预后的慢性淋巴水肿背景下发展,而RIA通常在辐射场中的放射治疗后数年发生。5在接受额外放射治疗的术后诱发慢性淋巴水肿的肿瘤患者中,LAS和RIA之间的确切区别尚不清楚。所有皮肤血管瘤的典型临床表现是一个扩大的弥漫性紫色斑块或结节,有时伴有溃疡。最初的病变可能会模仿瘀伤或感染,如丹毒,蜂窝织炎,酒渣鼻或肿胀狼疮,这可能会导致延误诊断。晚期血管肉瘤的预后通常较差,估计无进展生存时间为
Cutaneous angiosarcoma is a rare, aggressive malignant vascular neoplasm with a poor prognosis. 1, 2 Four main variants are currently recognised, namely; sporadic angiosarcoma (AS) of the scalp and face, lymphedema associated angiosarcoma (LAS), radiation-induced angiosarcoma (RIA) and the recently described rare, aggressive epithelioid subtype. 3, 4Among these four variants, angiosarcoma of the scalp and face, also known as Wilson–Jones angiosarcoma, senile angiosarcoma or malignant angioendothelioma is the most common. The disease mainly affects elderly individuals with a male predominance. LAS develops in the context of chronic lymphoedema after a mastectomy or other oncological or surgical interventions, whereas RIA occurs often years after radiotherapy in the radiation field. 5 In oncology patients with post-surgery induced chronic lymphoedema who have received additional radiation therapy, the exact differentiation between LAS and RIA is not clear. The typical clinical presentation of all cutaneous angiosarcomas is that of an enlarging diffuse violaceous plaque or nodule, at times with ulceration. Initial lesions may mimic bruising or infection such as erysipelas, cellulitis, rosacea or tumid lupus, which may result in delayed diagnosis. The prognosis for advanced angiosarcoma is generally poor with an estimated progression-free survival time of