Elawar A, Dandurand M, Jumez N, Delfour C, Branchereau P, Stoebner P, Meunier L
Service de dermatologie, hôpital Caremeau, CHU de Nîmes, place du professeur R.-Debré, 30029 Nîmes cedex 9, France.
Ann Dermatol Venereol. 2010 Feb;137(2):111-6. doi: 10.1016/j.annder.2009.11.008. Epub 2009 Dec 30.
Epithelioid angiosarcomas (EAS) of the aorta are a rare form of tumour usually diagnosed by histopathological analysis of the aorta. We report a case revealed by skin metastasis.
An 85-year-old man presented skin tumours associated with deterioration of his general condition and intense pain of the right lower limb. Physical examination showed three nodules of the lumbar area associated with an ipsilateral livedo extending to the right lower limb. The course of the disease involved distal ischaemia. Arterial ultrasound, aortography and CAT showed ectasia of the abdominal aorta with thrombosis and right subpopliteal occlusion. Histological examination of a nodule showed proliferation of malignant cells with expression of vimentin, CD 31, cytokeratins AE1/AE3 and cytokeratin 7. Stain for CD34 was negative. Histological investigation of the livedo showed a vascular embolus with epithelial-type cells positive for cytokeratin 7 and CD 31. The PET scan showed intense F-FDG uptake of the aorta extended to the iliac artery. Moreover, skin and osseous F-FDG uptake was seen. These findings suggested a diagnosis of EAS of the aorta with skin and osseous metastasis and vascular emboli.
Only 27 previous case reports of EAS based on appropriate immunohistochemical analysis have been published in the literature. These tumours typically arise in the abdominal aorta in association with metastasis in more than 80% of cases. Skin metastasis causes papular eruption, nodules and peripheral vascular disease. Embolic vascular occlusion results in ischaemia and in rare cases vasculitis. Our case report emphasizes four key points: the diagnostic value of an association of localized malignant skin tumours, extensive livedo, ipsilateral distal ischaemia, deterioration of the general condition and intense pain; the diagnostic value of endothelial markers, especially CD31, and potentially misleading co-expression of cytokeratin markers; in selected cases, additional imaging, such as PET scans, performed in our case for the first time prior to surgery of the aorta, may be helpful for the diagnosis of such neoplastic lesions of the aortic wall.
主动脉上皮样血管肉瘤（EAS）是一种罕见的肿瘤形式，通常通过主动脉的组织病理学分析来诊断。我们报告一例以皮肤转移为首发表现的病例。
一名85岁男性出现皮肤肿瘤，伴有全身状况恶化及右下肢剧痛。体格检查发现腰部有三个结节，同侧出现青斑并延伸至右下肢。疾病进展导致远端缺血。动脉超声、主动脉造影和计算机断层扫描（CAT）显示腹主动脉扩张伴血栓形成以及右腘下动脉闭塞。对一个结节进行组织学检查，结果显示恶性细胞增殖，波形蛋白、CD31、细胞角蛋白AE1/AE3和细胞角蛋白7呈阳性表达。CD34染色为阴性。对青斑进行组织学检查发现血管栓子，其中上皮样细胞细胞角蛋白7和CD31呈阳性。正电子发射断层扫描（PET）显示主动脉有强烈的氟代脱氧葡萄糖（F-FDG）摄取，并延伸至髂动脉。此外，皮肤和骨骼也有F-FDG摄取。这些发现提示诊断为主动脉EAS伴皮肤和骨骼转移以及血管栓子形成。
此前文献中仅发表了27例基于适当免疫组织化学分析的EAS病例报告。这些肿瘤通常发生在腹主动脉，超过80%的病例伴有转移。皮肤转移可导致丘疹样皮疹、结节和周围血管疾病。栓塞性血管闭塞会导致缺血，罕见情况下会引发血管炎。我们的病例报告强调了四个关键点：局部恶性皮肤肿瘤、广泛青斑、同侧远端缺血、全身状况恶化和剧痛同时出现的诊断价值；内皮标志物尤其是CD31的诊断价值，以及细胞角蛋白标志物可能产生误导的共表达情况；在某些病例中，额外的影像学检查，如我们在该病例中首次在主动脉手术前进行的PET扫描，可能有助于诊断此类主动脉壁肿瘤性病变。
