Wassef M, Vanwijck R, Clapuyt P, Boon L, Magalon G
Assistance publique-Hôpitaux de Paris, hôpital Lariboisière, laboratoire Jean-Roujeau, 2, université Paris-VII-René-Descartes, faculté de médecine, France.
Ann Chir Plast Esthet. 2006 Aug-Oct;51(4-5):263-81. doi: 10.1016/j.anplas.2006.07.017. Epub 2006 Sep 26.
The understanding of vascular anomalies (vascular tumours and vascular malformations) was obscured, for a long time, by confusion and uncertainties in nosology and terminology. The International Society for the Study of Vascular Anomalies (ISSVA) recently adopted a classification scheme, clearly separating vascular tumours (hemangiomas of different types) which result from active cell proliferation, from vascular malformations, which are inborn defects in vascular morphogenesis. These two types of lesions have different clinical behaviour and require different diagnostic and therapeutic strategies. The most frequent vascular tumour is infantile hemangioma. Its clinical aspects and evolution are well-known. New data have been recently obtained concerning the phenotype of tumour cells and its histogenesis. Of the numerous new vascular tumours, which have been recently described, only the congenital hemangiomas, the vascular tumours associated with the Maffucci syndrome and the tumours that may be complicated by a profound thrombocytopenia (Kasabach and Merritt phenomenon) will be considered. Vascular malformations can be classified according to the vessel(s) types they are composed of. A classification table is presented, separating the malformations of vascular trunks from tissular malformations which are more intimately embedded in the surrounding tissues. The different syndromes associated with vascular anomalies take also place in this table. The clinical, imaging and histological aspects of the most frequent malformations (capillary, venous, lymphatic and arteriovenous) are presented. This classification intend to clarify the nosology and terminology of the complex field of vascular tumours and malformation and to offer a common language to the different physicians and specialists contributing, preferably with a interdisciplinary approach, to the diagnosis and treatment of these difficult lesions.
长期以来,由于疾病分类学和术语方面的混乱与不确定性,对血管异常(血管肿瘤和血管畸形)的认识一直较为模糊。国际血管异常研究学会(ISSVA)最近采用了一种分类方案,明确区分了由活跃细胞增殖导致的血管肿瘤(不同类型的血管瘤)和血管畸形,后者是血管形态发生的先天性缺陷。这两种类型的病变具有不同的临床行为,需要不同的诊断和治疗策略。最常见的血管肿瘤是婴儿血管瘤。其临床特征和演变过程已为人熟知。最近在肿瘤细胞表型及其组织发生方面获得了新的数据。在最近描述的众多新型血管肿瘤中,仅考虑先天性血管瘤、与马富西综合征相关的血管肿瘤以及可能并发严重血小板减少症(卡萨巴赫-梅里特现象)的肿瘤。血管畸形可根据其组成的血管类型进行分类。文中给出了一个分类表,将血管主干畸形与更紧密嵌入周围组织的组织畸形区分开来。与血管异常相关的不同综合征也列于该表中。文中介绍了最常见畸形(毛细血管型、静脉型、淋巴管型和动静脉型)的临床、影像学和组织学特征。这种分类旨在阐明血管肿瘤和畸形这一复杂领域的疾病分类学和术语,并为不同的医生和专家提供一种通用语言,他们最好采用跨学科方法,共同参与这些疑难病变的诊断和治疗。