Fourth Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.
Anticancer Res. 2020 Feb;40(2):625-633. doi: 10.21873/anticanres.13992.
Primary angiosarcoma of the kidney is a rare tumor, hence little is known concerning its diagnostic features and therapeutic management. We conducted this survey to present a complete literature review with emphasis on clinicopathological features, diagnosis and therapy. A thorough search was conducted in MEDLINE/PubMed. All relevant studies concerning primary renal angiosarcomas in adults were thoroughly reviewed. Primary renal angiosarcoma is characterized by an overall poor prognosis, is of unknown etiology and occurs most commonly in males between 60 and 70 years old. Presence of distant metastasis at the time of diagnosis is prevalent. Histopathological examination and immunohistochemical studies are the most important diagnostic tools. Treatment options include surgery, chemotherapy, radiotherapy and immunotherapy. Conclusion: Primary renal angiosarcoma is a rare but aggressive malignancy with low response to available therapeutic regimens and dismal survival rates.
原发性肾血管肉瘤是一种罕见的肿瘤，因此，人们对其诊断特征和治疗管理知之甚少。我们进行了这项调查，旨在对其进行全面的文献回顾，重点介绍临床病理特征、诊断和治疗。在 MEDLINE/PubMed 上进行了彻底的搜索。对所有关于成人原发性肾血管肉瘤的相关研究进行了全面审查。原发性肾血管肉瘤预后总体较差，病因不明，最常见于 60 至 70 岁的男性。诊断时存在远处转移较为常见。组织病理学检查和免疫组织化学研究是最重要的诊断工具。治疗选择包括手术、化疗、放疗和免疫治疗。结论：原发性肾血管肉瘤是一种罕见但侵袭性的恶性肿瘤，对现有治疗方案反应不佳，生存率低。
