Vanbinst Anne-Marie, Brussaard Carola, Vergauwen Evelynn, Van Velthoven Vera, Kuijpers Robert, Michel Olaf, Foulon Ina, Jansen Anna C, Lefevere Bieke, Bohler Susanne, Keymolen Kathelijn, de Mey Johan, Michielsen Dirk, Andreescu Corina E, Gläsker Sven
1Department of Radiology, Universitair Ziekenhuis Brussel, Brussels, Belgium.
2Department of Neurosurgery, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Brussels, Belgium.
Hered Cancer Clin Pract. 2019 Jul 29;17:22. doi: 10.1186/s13053-019-0121-9. eCollection 2019.
Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited tumor syndrome. Affected patients develop central nervous system hemangioblastomas and abdominal tumors, among other lesions. Patients undergo an annual clinical screening program including separate magnetic resonance imaging (MRI) of the brain, whole spine and abdomen. Consequently, patients are repeatedly subjected to time-consuming and expensive MRI scans, performed with cumulative Gadolinium injections. We report our experience with a 35-min whole body MRI screening protocol, specifically designed for detection of VHL-associated lesions.
We designed an MRI protocol dedicated to the typical characteristics of VHL-associated lesions in different imaging sequences, within the time frame of 35 min. Blank imaging of the abdomen is carried out first, followed by abdominal sequences with Gadolinium contrast. Next, the full spine is examined, followed by imaging of the brain. A single dose of contrast used for abdominal imaging is sufficient for further highlighting of spine- and brain lesions, thus limiting the Gadolinium dosage. We used 1.5 Tesla equipment, dealing with fewer artifacts compared to a 3 Tesla system for spine- and abdominal imaging, while preserving acceptable quality for central nervous system images. In addition, imaging on a 1.5 Tesla scanner is slightly faster.
From January 2016 to November 2018, we performed 38 whole body screening MRIs in 18 VHL patients; looking for the most common types of VHL lesions in the abdomen, spine, and brain, both for new lesions and follow-up. The one-step approach MRI examinations lead to 6 surgical interventions for clinically significant or symptomatic hemangioblastomas in the brain and spine. One renal cell carcinoma was treated with radiofrequency ablation. In comparison with previous conventional MRI scans of the same patients, all lesions were visible with the focused protocol.
Annual screening in VHL disease can be done in a rapid, safe and sensitive way by using a dedicated whole body MRI protocol; saving MRI examination time and limiting Gadolinium dose.
冯·希佩尔-林道（VHL）病是一种常染色体显性遗传肿瘤综合征。受影响的患者会出现中枢神经系统血管母细胞瘤和腹部肿瘤等病变。患者每年接受一次临床筛查项目，包括分别对脑部、全脊柱和腹部进行磁共振成像（MRI）检查。因此，患者需要反复接受耗时且昂贵的MRI扫描，并累积注射钆造影剂。我们报告了我们使用专门设计用于检测VHL相关病变的35分钟全身MRI筛查方案的经验。
我们在35分钟的时间范围内，针对不同成像序列中VHL相关病变的典型特征设计了一种MRI方案。首先对腹部进行空白成像，然后进行钆造影剂增强的腹部序列成像。接下来检查全脊柱，随后对脑部进行成像。用于腹部成像的单剂量造影剂足以进一步突出脊柱和脑部病变，从而限制钆的用量。我们使用1.5特斯拉设备，与3特斯拉系统相比，在脊柱和腹部成像时产生的伪影较少，同时保持中枢神经系统图像的可接受质量。此外，在1.5特斯拉扫描仪上成像稍快一些。
从2016年1月至2018年11月，我们对18例VHL患者进行了38次全身筛查MRI，以寻找腹部、脊柱和脑部最常见类型的VHL病变，包括新病变和进行随访。这种一步式MRI检查方法导致对脑部和脊柱中具有临床意义或有症状的血管母细胞瘤进行了6次手术干预。1例肾细胞癌接受了射频消融治疗。与之前对同一患者进行的传统MRI扫描相比，所有病变在这种针对性方案下均可见。
通过使用专门的全身MRI方案，可以快速、安全且灵敏地对VHL病进行年度筛查，节省MRI检查时间并限制钆的剂量。
