遗传性肾癌的治疗策略:当前的建议和更新。

IF 2 4区 医学 Q3 MEDICINE, RESEARCH & EXPERIMENTAL Discovery medicine Pub Date : 2022-11-01
Shiva Singh, Aditi Chaurasia, Nikhil Gopal, Ashkan Malayeri, Mark W Ball
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引用次数: 0

摘要

一部分肾肿瘤(5-8%)与以下综合征相关:von Hippel-Lindau (VHL)综合征、birt - hogg - dub综合征(BHD)、结节性硬化症(TSC)、遗传性乳头状肾癌(HPRC)、遗传性平滑肌瘤病和肾细胞癌综合征(HLRCC)、BRCA1相关蛋白(BAP1)肿瘤易感综合征、丁二酸脱氢酶RCC (SDHB/C/D)。这些综合征有其特定的基因改变和相关的外部表现。由于这些综合征中肿瘤的组织病理学和侵袭性不同,管理策略可以从主动监测到预先手术切除。这篇综述描述了最常见的家族性肾癌综合征的具体特征,并讨论了当前的管理策略。
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Treatment Strategies for Hereditary Kidney Cancer: Current Recommendations and Updates.

A subset of renal tumors (5-8%) are associated with syndromes such as von Hippel-Lindau (VHL) syndrome, Birt-Hogg-Dubé syndrome (BHD), tuberous sclerosis complex (TSC), hereditary papillary renal carcinoma (HPRC), hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC), and BRCA1 associated protein (BAP1) tumor predisposition syndrome, succinate dehydrogenase RCC (SDHB/C/D). These syndromes have their specific defined genetic alterations and associated extrarenal manifestations. Due to varying histopathology and aggressiveness of the tumors amongst these syndromes, the management strategies can range from active surveillance to upfront surgical resection. This review delineates specific characteristics of the most common familial renal cancer syndromes and discusses current management strategies.

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来源期刊
Discovery medicine
Discovery medicine MEDICINE, RESEARCH & EXPERIMENTAL-
CiteScore
5.40
自引率
0.00%
发文量
80
审稿时长
6-12 weeks
期刊介绍: Discovery Medicine publishes novel, provocative ideas and research findings that challenge conventional notions about disease mechanisms, diagnosis, treatment, or any of the life sciences subjects. It publishes cutting-edge, reliable, and authoritative information in all branches of life sciences but primarily in the following areas: Novel therapies and diagnostics (approved or experimental); innovative ideas, research technologies, and translational research that will give rise to the next generation of new drugs and therapies; breakthrough understanding of mechanism of disease, biology, and physiology; and commercialization of biomedical discoveries pertaining to the development of new drugs, therapies, medical devices, and research technology.
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