肾髓质癌：拓扑异构酶抑制治疗的分子、病理和临床证据。
Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase-inhibiting therapy.
机构信息
The James Buchanan Brady Urologic Institute, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA.
出版信息
BJU Int. 2010 Jul;106(1):62-5. doi: 10.1111/j.1464-410X.2009.09139.x. Epub 2009 Dec 11.
STUDY TYPE
Aetiology (case series) Level of Evidence 4.
OBJECTIVE
To present the molecular rationale and potential clinical benefit of topoisomerase II (TopoII)-inhibiting therapy for renal medullary carcinoma (RMC), a rare but extremely lethal form of kidney cancer that classically afflicts young men with sickle-cell trait. The current therapeutic approach with these aggressive tumours is radical nephrectomy followed by systemic chemotherapy, but the prognosis remains dismal.
MATERIALS AND METHODS
The whole-genome expression was analysed in four RMC tumours. We also report a case of metastatic RMC in which a complete response was achieved for 9 months using a TopoII-inhibiting therapy.
RESULTS
Expanded whole-genome expression analysis showed increases of TopoII in all cases. There was also overall deregulation of DNA remodelling and repair, and an ontological association between RMC and urothelial carcinoma. Using a TopoII-inhibiting agent, there was a complete response for 9 months in a patient with metastatic RMC.
CONCLUSION
This report provides molecular evidence for the rational use of TopoII inhibitors in the treatment of RMC.
研究类型
病因学（病例系列）证据水平 4。
目的
介绍拓扑异构酶 II（TopoII）抑制剂治疗肾髓质癌（RMC）的分子原理和潜在临床益处。RMC 是一种罕见但极其致命的肾癌形式，经典地影响带有镰状细胞特征的年轻男性。目前对这些侵袭性肿瘤的治疗方法是根治性肾切除术加全身化疗，但预后仍然很差。
材料和方法
对四个 RMC 肿瘤进行了全基因组表达分析。我们还报告了一例转移性 RMC 病例，该病例使用 TopoII 抑制剂治疗 9 个月后获得完全缓解。
结果
扩展的全基因组表达分析显示所有病例中 TopoII 均增加。DNA 重塑和修复的整体失调，以及 RMC 与尿路上皮癌之间的本体关联。使用 TopoII 抑制剂，转移性 RMC 患者的完全缓解持续了 9 个月。
结论
本报告为 TopoII 抑制剂在 RMC 治疗中的合理应用提供了分子证据。
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