MP80-13 RISK FACTORS FOR SURVIVAL IN PATIENTS WITH VON HIPPEL-LINDAU DISEASE: A LARGE SINGLE-CENTER RETROSPECTIVE STUDY OF 734 VHL PATIENTS

Abstract

You have accessJournal of UrologyKidney Cancer: Epidemiology & Evaluation/Staging/Surveillance III (MP80)1 Apr 2020MP80-13 RISK FACTORS FOR SURVIVAL IN PATIENTS WITH VON HIPPEL-LINDAU DISEASE: A LARGE SINGLE-CENTER RETROSPECTIVE STUDY OF 734 VHL PATIENTS Kaifang Ma*, Lei Li, Lin Cai, and Kan Gong Kaifang Ma*Kaifang Ma* More articles by this author , Lei LiLei Li More articles by this author , Lin CaiLin Cai More articles by this author , and Kan GongKan Gong More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000000972.013AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Von Hippel-Lindau (VHL) disease is an autosomal-dominant hereditary cancer syndrome, which characterized by a poor prognosis. Currently, the risk factors for VHL survival remain unclear. This study aimed to investigate whether VHL survival is influenced by hereditary and clinical factors. METHODS: In this retrospective study, we recruited 734 patients from 372 VHL families. Kaplan-Meier plot and Cox regression model were used to evaluate the median survival and assess how survival was influenced by sex, birth year, family history, clinical type, mutation type, onset age and initial symptom. Age-related risks of VHL-associated tumors and patient survival were compared. RESULTS: The median survival for Chinese patients with VHL disease was 61 years. Patients with positive family history, early-onset age and truncating mutation types had poorer overall and VHL-related survival. Patients with positive family history (HR:2.32, p=0.014) and early-onset age (HR:3.79, p < 0.001) were related to a higher risk of death from central nervous system haemangioblastoma. CONCLUSIONS: This largest singer-center retrospective research indicated that onset age, family history, mutation type and initial symptom have an effect on the survival of patients with VHL disease, which is beneficial to genetic counselling and clinical decision-making. Source of Funding: This work was supported by the National Natural Science Foundation of China (grant 81572506 and grant 81872081). © 2020 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 203Issue Supplement 4April 2020Page: e1230-e1230 Advertisement Copyright & Permissions© 2020 by American Urological Association Education and Research, Inc.MetricsAuthor Information Kaifang Ma* More articles by this author Lei Li More articles by this author Lin Cai More articles by this author Kan Gong More articles by this author Expand All Advertisement PDF downloadLoading ...