Oliveira Monteiro Ana, Branco Ferreira Inês, César Artur, Mota Alberto, Pinheiro Jorge, Lopes José Manuel, Cardoso M Teresa
Department of Internal Medicine, Centro Hospitalar Universitário de S. João, Porto, Portugal.
Department of Dermatology and Venereology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Porto, Portugal.
Eur J Case Rep Intern Med. 2020 Sep 25;7(12):001939. doi: 10.12890/2020_001939. eCollection 2020.
Cutaneous angiosarcoma is a rare, highly malignant tumour of vascular endothelial origin. It usually arises in the skin and superficial soft tissue, mostly on the head and neck. It presents as a variety of lesions, and so is considered a great mimicker, leading to a delay in diagnosis and evidencing the importance of biopsy with immunohistochemistry confirmation. There are few reports of extremity involvement in patients with pre-existing chronic lymphoedema, or exposure to radiation therapy. We report the case of an 82-year-old woman with lower limb extensive cutaneous involvement, distant metastatic disease, and poor therapy response. Its rare location without predisposing factors highlights the need to raise awareness about this disease.
Extremity involvement of cutaneous angiosarcoma has been rarely described. The marked heterogeneity in presentation leads to a delay in diagnosis and poor prognosis, so the index of suspicion should be high.The cases reported in the literature describe a well-known relationship between cutaneous angiosarcoma and predisposing factors, but its absence should not exclude the diagnosis.This case highlights the importance of recognizing and biopsy suspected skin lesions for immunohistochemistry diagnostic confirmation.
皮肤血管肉瘤是一种罕见的、高度恶性的血管内皮源性肿瘤。它通常发生于皮肤和浅表软组织,多位于头颈部。它表现为多种病变,因此被认为是一种极易造成误诊的疾病,导致诊断延迟,并证明了活检及免疫组化确诊的重要性。关于既往存在慢性淋巴水肿或接受过放射治疗的患者出现肢体受累的报道很少。我们报告了一例82岁女性患者,其下肢广泛皮肤受累、有远处转移疾病且治疗反应不佳。其罕见的发病部位且无 predisposing factors(此处可能有误,推测应为“易感因素”之类的词)凸显了提高对该疾病认识的必要性。
皮肤血管肉瘤累及肢体的情况鲜有描述。其表现的显著异质性导致诊断延迟且预后不良,因此怀疑指数应较高。文献报道的病例描述了皮肤血管肉瘤与易感因素之间的一种众所周知的关系,但无易感因素不应排除诊断。该病例凸显了识别并对疑似皮肤病变进行活检以通过免疫组化进行诊断确认的重要性。