Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases.
Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases.
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DOI：
10.3978/j.issn.2223-4683.2015.05.04
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发表时间：
2015-08
影响因子：
2
通讯作者：
Omiyale AO
中科院分区：
文献类型：
--
作者：
Omiyale AO
血管肉瘤（AS）是一种恶性肿瘤，发生率低于2%的软组织肉瘤。原发于肾脏的肿瘤很少见，其发病机制在很大程度上仍不清楚，并且与其他肾脏肿瘤有重叠的特征。本文的目的是回顾文献中原发性肾动脉硬化的病例报告。检索词为原发性肾脏AS、原发性肾脏AS和原发性肾脏血管肉瘤。审查的病例总数为62例。平均发病年龄为61岁，男性多见。诊断时转移性疾病占报告病例的44.9%（22/49），44.4%（12/27）诊断时无转移性疾病的患者随后发生转移。原发性肾脏动脉硬化症是一种罕见的恶性肿瘤，预后差。局部复发和远处转移是常见的。原发性肾脏AS与其他肾脏肿瘤的临床表现相似，影像学无法进行肿瘤特异性诊断。组织学检查和免疫组化对确诊有重要意义。目前的治疗方案包括手术、放疗和化疗的可变组合。
Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.