Review of renal anastomosing hemangioma with focus on clinical and pathological aspects.
Review of renal anastomosing hemangioma with focus on clinical and pathological aspects.
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DOI：
10.5114/pjp.2016.61443
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发表时间：
2016-08
期刊：
影响因子：
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通讯作者：
N. Kuroda;C. Ohe;S. Deepika;K. Yorita;S. Mikami;M. Furuya;Y. Nagashima;O. Hes;A. Agaimy;M. Michal;M. Amin
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文献类型：
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作者：
N. Kuroda;C. Ohe;S. Deepika;K. Yorita;S. Mikami;M. Furuya;Y. Nagashima;O. Hes;A. Agaimy;M. Michal;M. Amin
肾吻合性血管瘤(RAH)是最近提出的一种新的实体。在本文中，我们总结了该肿瘤的临床病理特征。RAH通常发生在终末期肾脏疾病的背景下。肉眼可见肿瘤边界清楚，切面显示红木棕色海绵组织，中心位于肾髓质。肿瘤通常很小，但报道的是较大的病变。镜下可见肿瘤内有血窦状血管通道，内有立方内皮细胞，偶见内皮细胞呈鞋钉样外观，与脾血窦相似。肿瘤内皮细胞胞浆内可见嗜酸性透明小球。血管腔内可见髓外造血，内含红系前体细胞和巨核细胞。免疫组织化学显示内皮细胞CD31、CD34阳性，D2-40、GLUT-1、HHV8阴性。内皮细胞周围的间质对的平滑肌作用呈阳性反应。到目前为止，还没有关于RAH分子遗传学方面的研究。根据病变的部位和大小，这种肿瘤是无痛的，部分或肾切除就足够作为一种治疗方式。
Renal anastomosing hemangiomas (RAH) has been recently proposed as a new entity. In this article, we summarize the clinicopathologic features of this tumor. RAH usually develops on a background of end-stage renal disease. Macroscopically, tumors are well-defined and their cut surface shows mahogany brown spongy tissue with epicenter in the renal medulla. Tumors are usually small, but larger lesions are reported. On microscopic examination, the tumor consists of sinusoid-like vascular channels lined by cuboidal endothelial cells with occasional hobnail-like appearance of endothelial cells closely mimicking splenic sinusoids. Eosinophilic hyaline globules may be present in the cytoplasm of neoplastic endothelial cells. Extramedullary hematopoiesis containing erythroid precursor and megakaryocytes may be present in the vascular lumens. Immunohistochemically, endothelial cells are positive for CD31 and CD34, but negative for D2-40, GLUT-1 and HHV8. The surrounding stroma around endothelial cells demonstrates positivity for smooth muscle action. To date, there are no studies on molecular genetic aspects of RAH. This tumor is indolent based on site and size of the lesion, partial or nephrectomy is sufficient as a therapeutic modality.