Department of Radiology, Izmir Atatürk Education and Research Hospital, Izmir, Turkey.
Radiol Oncol. 2010 Sep;44(3):164-7. doi: 10.2478/v10019-010-0014-z. Epub 2010 Sep 9.
Von Hippel Lindau disease is an autosomal dominant multisystem/multitumoral cancer disease diagnosed by clinical, radiologic and genetic findings. Its prevalence has been estimated to be of 1/36000 inhabitants. The tumours can be benign or malignant.
We represent MR findings of a family with ten children. Mother and five siblings had von Hippel-Lindau disease.
Radiologic imaging is very important for the early diagnosis and treatment of asymptomatic patients. Diagnosing it early is important because the tumours in von Hippel Lindau disease are treatable. Also, an early detection allows the patient's survival and quality of life. A multidisciplinary team approach is important in screening.
von Hippel-Lindau 病是一种常染色体显性遗传的多系统/多肿瘤性疾病，通过临床、影像学和遗传学发现进行诊断。其患病率估计为每 36000 人中有 1 例。肿瘤可以是良性的也可以是恶性的。
我们介绍了一个有十个孩子的家庭的磁共振成像结果。母亲和五个兄弟姐妹患有 von Hippel-Lindau 病。
影像学检查对无症状患者的早期诊断和治疗非常重要。早期诊断很重要，因为 von Hippel-Lindau 病中的肿瘤是可以治疗的。此外，早期发现可以提高患者的生存率和生活质量。多学科团队在筛查中很重要。
