PD52-08 ANALYSIS OF RISK FACTORS FOR SURVIVAL AND PROGNOSIS OF PATIENTS WITH VON HIPPEL-LINDAU SYNDROME: A LARGE RETROSPECTIVE STUDY
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You have accessJournal of UrologyKidney Cancer: Epidemiology & Evaluation/Staging/Surveillance II (PD52)1 Sep 2021PD52-08 ANALYSIS OF RISK FACTORS FOR SURVIVAL AND PROGNOSIS PATIENTS WITH VON HIPPEL-LINDAU SYNDROME: A LARGE RETROSPECTIVE STUDY Kaifang Ma, Kenan Zhang, Jingcheng Zhou, Lin Cai, and Kan Gong MaKaifang Ma More articles by this author , ZhangKenan Zhang ZhouJingcheng Zhou CaiLin Cai GongKan View All Author Informationhttps://doi.org/10.1097/JU.0000000000002079.08AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION OBJECTIVE: Von Hippel-Lindau (VHL) disease is an autosomal-dominant hereditary cancer syndrome, which characterized a poor prognosis. Renal cell carcinoma central nervous system hemangioblastoma are the most common causes death in VHL disease. Currently, risk factors for survival remain unclear. This study aimed investigate whether influenced clinical factors. METHODS: In retrospective study, we recruited 776 patients from 304 families. Kaplan-Meier plot Cox regression model were used evaluate median assess how was sex, birth year, family history, type, mutation onset age initial symptom. Age-related risks VHL-associated tumors patient compared. RESULTS: The Chinese with 61 years. Patients positive early-onset truncating types had poorer overall VHL-related survival. history (HR:2.32, p=0.014) (HR:3.79, p<0.001) related higher hemangioblastoma. CONCLUSIONS: largest singer-center research indicated that age, type symptom effect on disease, beneficial genetic counselling decision-making. Source Funding: funded National Natural Science Foundation China (No. 81572506 81872081) © 2021 American Urological Association Education Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e913-e914 Advertisement Copyright Permissions© Inc.MetricsAuthor Information Expand Loading ...
منابع مشابه
Manifestations of Von Hippel Lindau syndrome: a retrospective national review.
INTRODUCTION Von Hippel Lindau (VHL) disease is a syndrome that is defined by variety of tumours such as cerebellar haemangioblastomas, renal cell carcinomas, phaeochromocytomas, pancreatic adenomas and ear, nose and throat (ENT) adenomas. This disease is often genetic and inherited in an autosomal dominant fashion, and can present in childhood, adolescence or adult life. This study describes t...
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Von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome characterized by the development of multiple vascular tumours. The syndrome is caused by inactivation of the VHL protein (pVHL) and increased production of VEGF, PDGF, and TGF-α. The course of VHL syndrome is associated with the development of multiple vascular tumours. Most frequently, these include retinal and central nervous sys...
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Von Hippel-Lindau syndrome (VHL) is a familial neoplastic condition seen in approximately 1 in 36,000 live births. It is caused by germline mutations of the tumor suppressor gene VHL, located on the short arm of chromosome 3. While the majority of the affected individuals have a positive family history, up to 20% of cases arise from de novo mutations. VHL syndrome is characterized by the presen...
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We report an unusual case of a young male with cerebellar hemangioblastoma treated previously for medullary carcinoma of thyroid, whose PET/CT scans revealed a constellation of findings that suggested the rare Von Hippel Lindau syndrome. The diagnosis was clinched by confirming the findings on whole body contrast enhanced computed tomography (CECT) and contrast enha...
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ژورنال
عنوان ژورنال: The Journal of Urology
سال: 2021
ISSN: ['0022-5347', '1527-3792']
DOI: https://doi.org/10.1097/ju.0000000000002079.08