JAMA Oncology ( IF 22.5 ) Pub Date : 2018-01-01 , DOI: 10.1001/jamaoncol.2017.3428 Amit Tirosh 1 , Samira M Sadowski 2 , W Marston Linehan 3 , Steven K Libutti 4, 5 , Dhaval Patel 6 , Naris Nilubol 6 , Electron Kebebew 6
|
Von Hippel–Lindau (VHL) disease is a familial cancer syndrome caused by a germline mutation in the VHL tumor suppressor gene (OMIM 193300).1 The prevalence of pancreatic neuroendocrine tumors (PNETs) in VHL disease ranges from 9% to 17% and is characterized by a better prognosis compared with sporadic PNETs.2,3 Several risk factors for PNET metastasis in VHL disease have been reported4 but have not been validated. We conducted a prospective study (NCT00062166) to evaluate the natural history of VHL disease–associated pancreatic lesions to determine what factors were associated with PNET phenotype and prognosis.
中文翻译：
von Hippel-Lindau病患者的VHL基因型与胰腺神经内分泌肿瘤表型的相关性。
冯·希佩尔·林道（VHL）病是由VHL肿瘤抑制基因（OMIM 193300）的种系突变引起的家族性癌症综合症。1在VHL疾病中，胰腺神经内分泌肿瘤（PNET）的患病率为9％至17％，与散发性PNET相比，其预后较好。2 ，3在VHL病的原始神经外胚层肿瘤转移一些危险因素已报告4，但尚未得到证实。我们进行了一项前瞻性研究（NCT00062166），以评估VHL疾病相关的胰腺病变的自然史，以确定哪些因素与PNET表型和预后相关。
京公网安备 11010802027423号