Scholarlink

<p>von Hippel-Lindau (VHL) disease is a rare tumor syndrome involving neoplasms in multiple organs. Despite extensive research in Western countries, large-scale studies in East Asia remain limited. To address this gap, we conducted a nationwide retrospective cohort study evaluating the incidence, prevalence, survival, comorbidities, and manifestations of VHL disease in Korea. This study utilized authorized data from the Korean National Health Insurance Service (NHIS) cohort database. Patients with VHL disease were identified between January 1, 2009, and December 31, 2022. Annual population data for Korea during the study period were obtained from the resident registration central population records. Among approximately 50&#xa0;million Koreans, 390 patients with VHL disease were diagnosed during the study period. The mean age at diagnosis was 34.5 ± 14.8 years, with 30 deaths (7.7%) during the cohort period. The age-standardized annual incidence (2011–2022) was 0.52 per 1,000,000 and the prevalence in 2022 was 7.02 per 1,000,000. The 5- and 10-year relative survival rates were 0.924 and 0.874 for males, and 0.982 and 0.931 for females, respectively. All investigated comorbidities, including all neoplasms, showed increasing incidence over time. Cerebellar hemangioblastoma was associated with the highest cancer-related mortality risk (OR, 5.436). This first nationwide analysis of VHL disease in Korea outlines its epidemiological and clinical characteristics, providing insights into the epidemiological and clinical heterogeneity of VHL disease.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Epidemiology and clinical outcomes of von Hippel-Lindau disease in Korea: a nationwide cohort study

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年7月14日

查看全文
  • Seung Woo Choi,
  • Sang-Jun Shin,
  • Kwangsic Joo,
  • Sangchul Lee,
  • Se Hyun Kim,
  • Sang Jun Park,
  • Jeongwoo Lee,
  • Se Joon Woo

摘要

von Hippel-Lindau (VHL) disease is a rare tumor syndrome involving neoplasms in multiple organs. Despite extensive research in Western countries, large-scale studies in East Asia remain limited. To address this gap, we conducted a nationwide retrospective cohort study evaluating the incidence, prevalence, survival, comorbidities, and manifestations of VHL disease in Korea. This study utilized authorized data from the Korean National Health Insurance Service (NHIS) cohort database. Patients with VHL disease were identified between January 1, 2009, and December 31, 2022. Annual population data for Korea during the study period were obtained from the resident registration central population records. Among approximately 50 million Koreans, 390 patients with VHL disease were diagnosed during the study period. The mean age at diagnosis was 34.5 ± 14.8 years, with 30 deaths (7.7%) during the cohort period. The age-standardized annual incidence (2011–2022) was 0.52 per 1,000,000 and the prevalence in 2022 was 7.02 per 1,000,000. The 5- and 10-year relative survival rates were 0.924 and 0.874 for males, and 0.982 and 0.931 for females, respectively. All investigated comorbidities, including all neoplasms, showed increasing incidence over time. Cerebellar hemangioblastoma was associated with the highest cancer-related mortality risk (OR, 5.436). This first nationwide analysis of VHL disease in Korea outlines its epidemiological and clinical characteristics, providing insights into the epidemiological and clinical heterogeneity of VHL disease.