Omiyale Ayo O, Carton James
Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK.
Curr Urol Rep. 2018 Jan 31;19(2):4. doi: 10.1007/s11934-018-0755-6.
Primary angiosarcoma of the kidney is extremely rare; hence, relatively little is known regarding its clinicopathologic features and prognosis. Herein, we review the literature on primary renal angiosarcoma with emphasis on the clinical and pathologic features.
Approximately 64 cases have been reported in the literature, and most cases occur in the 6th-7th decade with a strong male predominance. The aetiology is unknown. Patients present with flank pain, haematuria, abdominal mass and weight loss. A considerable number of patients develop metastatic disease at diagnosis or shortly afterwards. Grossly, the tumour comprises ill-defined haemorrhagic spongy masses often with necrosis. Microscopically, the tumour is composed of anastomosing capillary-sized vessels which are lined by malignant endothelial cells. The mainstay of treatment is surgery followed by radiation therapy with or without chemotherapy. Renal angiosarcomas are highly aggressive tumours with dismal outcome, and they must be distinguished from morphologically similar lesions of the kidney.
肾原发性血管肉瘤极为罕见；因此，关于其临床病理特征和预后了解相对较少。在此，我们回顾原发性肾血管肉瘤的文献，重点关注临床和病理特征。
文献报道约64例，大多数病例发生在6至7十年代，男性占主导。病因不明。患者表现为侧腹痛、血尿、腹部肿块和体重减轻。相当数量的患者在诊断时或之后不久发生转移。大体上，肿瘤由边界不清的出血性海绵状肿块组成，常伴有坏死。显微镜下，肿瘤由相互吻合的毛细血管大小的血管组成，内衬恶性内皮细胞。治疗的主要方法是手术，随后进行有或无化疗的放疗。肾血管肉瘤是侵袭性很强的肿瘤，预后不佳，必须与形态学上相似的肾脏病变相鉴别。
