Lach Boleslaw, Hassounah Maher, Khafaga Yasser
Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Fetal Pediatr Pathol. 2008;27(3):175-83. doi: 10.1080/15513810802216002.
We describe a pediatric case of primary angiosarcoma of the brain displaying striking intravascular papillary pattern, consistent with the "Dabska tumor," often in continuity with a massive, multifocal intravascular papillary endothelial hyperplasia. The tumor contained small hemangioma and obliterated dysplastic arteries as well as very large thin-walled veins. The surrounding brain tissue showed scattered telangiectasias, conglomerates of calcified dysplastic arteries, old hemorrhages and gliosis. Colocalization of these lesions suggests the development of a papillary angiosarcoma in the pre-existing vascular malformation. Although never reported, the possibility of a malignant transformation of endothelial papillary hyperplasia also should be considered in this case.
我们描述了一例小儿原发性脑血管肉瘤,其呈现出显著的血管内乳头状模式,与“达布斯卡瘤”一致,常与大量多灶性血管内乳头状内皮增生相连续。肿瘤包含小血管瘤、闭塞的发育异常动脉以及非常大的薄壁静脉。周围脑组织显示散在的毛细血管扩张、钙化的发育异常动脉团块、陈旧性出血和胶质增生。这些病变的共定位提示在先前存在的血管畸形中发生了乳头状血管肉瘤。尽管从未有过报道,但在这种情况下也应考虑内皮乳头状增生恶变的可能性。