Sudarshan Sunil, Linehan W Marston
Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892-1107, USA.
Semin Oncol. 2006 Oct;33(5):544-51. doi: 10.1053/j.seminoncol.2006.06.008.
Kidney cancer is not a single disease. It is made up of a number of different types of cancer that occur in the kidney, each with a different histologic type, having a different clinical course, responding differently to therapy and caused by a different gene. The identification of families with a predisposition to the development of renal neoplasms, including von Hippel-Lindau (VHL), hereditary papillary renal carcinoma (HPRC), Birt-Hogg-Dubé (BHD), and hereditary leiomyomatosis and renal cell cancer (HLRCC), has made possible the identification of the different genes for these cancers. The genetic basis for each of these has been identified with current investigation focusing on the mechanisms of carcinogenesis. The elucidation of molecular pathogenesis in these familial forms of kidney cancer should provide the opportunity to determine successful approaches for novel therapeutic agents.
肾癌并非单一疾病。它由多种发生于肾脏的不同类型癌症组成，每种癌症具有不同的组织学类型、不同的临床病程、对治疗的反应不同且由不同基因引起。对易患肾肿瘤的家族的识别，包括冯·希佩尔-林道（VHL）病、遗传性乳头状肾癌（HPRC）、比特-霍格-杜布é（BHD）综合征以及遗传性平滑肌瘤病和肾细胞癌（HLRCC），使得识别这些癌症的不同基因成为可能。目前已确定了每种癌症的遗传基础，研究重点在于致癌机制。阐明这些家族性肾癌的分子发病机制应能为确定新型治疗药物的成功方法提供契机。
