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首页> 外文期刊>International Journal of Surgical Oncology >Familial Renal Cancer: Molecular Genetics and Surgical Management
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Familial Renal Cancer: Molecular Genetics and Surgical Management

机译：家族性肾癌：分子遗传学和外科治疗

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摘要

Familial renal cancer (FRC) is a heterogeneous disorder comprised of a variety of subtypes. Each subtype is known to have unique histologic features, genetic alterations, and response to therapy. Through the study of families affected by hereditary forms of kidney cancer, insights into the genetic basis of this disease have been identified. This has resulted in the elucidation of a number of kidney cancer gene pathways. Study of these pathways has led to the development of novel targeted molecular treatments for patients affected by systemic disease. As a result, the treatments for families affected by von Hippel-Lindau (VHL), hereditary papillary renal carcinoma (HPRC), hereditary leiomyomatosis renal cell carcinoma (HLRCC), and Birt-Hogg-Dubé (BHD) are rapidly changing. We review the genetics and contemporary surgical management of familial forms of kidney cancer.
机译：家族性肾癌（FRC）是一种由多种亚型组成的异质性疾病。已知每种亚型都有独特的组织学特征，遗传改变和对治疗的反应。通过对受遗传性肾癌影响的家庭的研究，已经确定了对该疾病的遗传基础的见解。这导致阐明了许多肾癌基因途径。这些途径的研究已导致针对全身性疾病患者的新型靶向分子治疗的发展。结果，受冯·希佩尔·林道（VHL），遗传性乳头状肾癌（HPRC），遗传性平滑肌瘤肾细胞癌（HLRCC）和Birt-Hogg-Dubé（BHD）影响的家庭的治疗方法正在迅速改变。我们回顾了家族性形式的肾癌的遗传学和当代外科治疗。

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