Hereditary Cancer in Clinical Practice (Jul 2019)

A focused 35-minute whole body MRI screening protocol for patients with von Hippel-Lindau disease

  • Anne-Marie Vanbinst,
  • Carola Brussaard,
  • Evelynn Vergauwen,
  • Vera Van Velthoven,
  • Robert Kuijpers,
  • Olaf Michel,
  • Ina Foulon,
  • Anna C. Jansen,
  • Bieke Lefevere,
  • Susanne Bohler,
  • Kathelijn Keymolen,
  • Johan de Mey,
  • Dirk Michielsen,
  • Corina E. Andreescu,
  • Sven Gläsker

DOI
https://doi.org/10.1186/s13053-019-0121-9
Journal volume & issue
Vol. 17, no. 1
pp. 1 – 6

Abstract

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Abstract Background 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. Methods 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. Results 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. Conclusions 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.

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