Janmohamed Sherief R, Madern Gerard C, de Laat Peter C J, Oranje Arnold P
Department of Paediatric Surgery, Erasmus University Medical Centre, Dr. Molewaterplein 60, 3015 GJ, Rotterdam, The Netherlands,
Eur J Pediatr. 2015 Jan;174(1):97-103. doi: 10.1007/s00431-014-2403-6. Epub 2014 Aug 26.
Infantile haemangioma (IH) is the most frequent childhood tumour. Although it is benign and self-limiting, severe complications can arise due to localisation and fast tumour growth. Management and therapy of IH has changed greatly after 2008 with propranolol. However, the pathogenesis remains elusive. This update provides an overview of all possible mechanisms currently considered. We discuss the possibility that several mechanisms act together, although local hypoxia seems to be important. Clinically, in about half of the cases, an IH is preceded by an anaemic macula (local ischaemia) or a so-called precursor lesion. Laboratory findings indicate stabilisation and an increased transcription activity of hypoxia-inducible factor 1 alpha (HIF1α), leading to up-regulation of its downstream target genes (such as vascular endothelial growth factor (VEGF)), which normally occurs in cases of hypoxia.
Three main hypotheses have been proposed, namely (1) the theory of tissue hypoxia, (2) the theory of embolization of placental endothelial cells and (3) the theory of increased angiogenic and vasculogenic activity.
婴儿血管瘤(IH)是儿童期最常见的肿瘤。尽管它是良性且自限性的,但由于肿瘤的位置和快速生长仍可能出现严重并发症。2008年后,随着普萘洛尔的应用,婴儿血管瘤的管理和治疗发生了很大变化。然而,其发病机制仍不清楚。本综述概述了目前所考虑的所有可能机制。我们讨论了几种机制共同作用的可能性,尽管局部缺氧似乎很重要。临床上,约一半的病例中,婴儿血管瘤之前会出现贫血性斑(局部缺血)或所谓的前驱病变。实验室检查结果表明缺氧诱导因子1α(HIF1α)稳定且转录活性增加,导致其下游靶基因(如血管内皮生长因子(VEGF))上调,这通常发生在缺氧情况下。
提出了三个主要假说,即(1)组织缺氧理论,(2)胎盘内皮细胞栓塞理论,(3)血管生成和血管发生活性增加理论。