Roudier-Pujol C, Enjolras O, Lacronique J, Guillemette J, Herbreteau D, Leibowitch M, Escande J P
Service de Dermatologie, Hôpital Tarnier-Cochin, Paris.
Ann Dermatol Venereol. 1994;121(12):898-904.
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour of the soft tissue having an intermediate malignancy. Cutaneous presentations have exceptionally been reported.
A young woman, initially affected by an EHE with cutaneous and bone involvement, then with pulmonary and hepatic localizations, was in partial remission after one year of treatment with alpha 2a interferon.
In a critical review of the literature, we describe the main features of this tumour and the difficulties to classify this entity in the vascular tumors' spectrum. Histologically, epithelioid hemangioendothelioma has a very characteristic appearance and is clearly different from other vascular tumors as angiolymphoid hyperplasia with eosinophilia and Kimura's disease. The assumption of a multicentric or a metastatic origin when multiple tumor deposits are found, is still not elucidated. Different therapeutic approaches are proposed: surgery, interferon, interleukin-2, retinoids. Randomized studies would be necessary to confirm our results with alpha 2a interferon but are probably difficult to realize because of the lack of cases.
上皮样血管内皮瘤（EHE）是一种罕见的软组织血管肿瘤，具有中度恶性。皮肤表现的病例报道极为罕见。
一名年轻女性，最初患有累及皮肤和骨骼的EHE，随后出现肺部和肝脏病变，在接受α2a干扰素治疗一年后病情部分缓解。
在对文献的批判性综述中，我们描述了该肿瘤的主要特征以及在血管肿瘤谱系中对这一实体进行分类的困难。组织学上，上皮样血管内皮瘤具有非常典型的外观，明显不同于其他血管肿瘤，如伴有嗜酸性粒细胞增多的血管淋巴样增生和木村病。当发现多个肿瘤病灶时，关于其多中心起源或转移起源的假设仍未阐明。目前提出了不同的治疗方法：手术、干扰素、白细胞介素-2、维甲酸。需要进行随机研究以证实我们使用α2a干扰素的结果，但由于病例数量不足，可能难以实现。
