Victoria Martínez A M, Cubells Sánchez L, Esteve Martínez A, Estela Cubells J R, Febrer Bosch I, Alegre de Miquel V, Oliver Martínez V
Servicio de Dermatología, Consorcio Hospital General Universitario de Valencia, Valencia, España.
Servicio de Dermatología, Consorcio Hospital General Universitario de Valencia, Valencia, España.
An Pediatr (Barc). 2015 Sep;83(3):201-8. doi: 10.1016/j.anpedi.2014.10.018. Epub 2014 Nov 24.
Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood, although there are cases reported in adults. Clinical presentation and evolution of TA can vary. Histologically, it takes on a classic appearance of vascular tufts ("cannon ball" like appearance).
A retrospective observational study was conducted that included all patients diagnosed with TA at our center in the last 20 years.
A series of 9 cases of tufted angioma in childhood are presented, 77.7% of which were congenital. This represents a frequency higher than previously described. Spontaneous regression was observed in 55.5% of the cases, and was more frequent in the congenital TA group. Unlike other TA series reported in the literature, a higher proportion of patients with spontaneous regression was observed in this series, with a higher prevalence in females (6 out of 9 children) and predominantly located in the upper limbs. None of our patients had Kasabach-Merritt phenomenon.
There are many ways of treating TA, but none are uniformly effective. Given the high rate of spontaneous regression in congenital or early TA, we suggest that, in the absence of other complications, monitoring would be a good option for management.
簇状血管瘤(TA)是一种罕见的良性血管肿瘤,大多在婴儿期或儿童早期出现,不过也有成人病例的报道。TA的临床表现和病程可能有所不同。在组织学上,它呈现出血管簇的典型外观(“炮弹”样外观)。
进行了一项回顾性观察研究,纳入了过去20年在我们中心诊断为TA的所有患者。
呈现了一系列9例儿童簇状血管瘤病例,其中77.7%为先天性。这一频率高于先前报道。55.5%的病例观察到自发消退,且在先天性TA组中更常见。与文献中报道的其他TA系列不同,本系列中观察到自发消退的患者比例更高,女性患病率更高(9名儿童中有6名),且主要位于上肢。我们的患者均未出现卡萨巴赫-梅里特现象。
TA有多种治疗方法,但均非完全有效。鉴于先天性或早期TA的自发消退率较高,我们建议,在无其他并发症的情况下,监测将是一种良好的管理选择。