Central nervous system gadolinium accumulation in patients undergoing periodical contrast MRI screening for hereditary tumor syndromes

Evelynn Vergauwen,Dieter De Clerck,Peter Janssens,Michel Van Lint,Vera Van Velthoven,Anne C Houtman,Anna C Jansen,Susanne Bohler,Olaf Michel,Bieke Lefevere,Sven Gläsker,Anne-Marie Vanbinst,Kathelijn Keymolen,Dirk Michielsen,Carola Brussaard

doi:10.1186/s13053-017-0084-7

Evelynn Vergauwen, Dieter De Clerck + Show 13 more

Open Access

https://doi.org/10.1186/s13053-017-0084-7

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Abstract

BackgroundPatients with hereditary tumor syndromes undergo periodical magnetic resonance imaging (MRI) screening with Gadolinium contrast. Gadolinium accumulation has recently been described in the central nervous system after repeated administrations. The prevalence and rate of accumulation in different subgroups of patients are unknown. Neither are the mechanism nor clinical impact. This may cause uncertainty about the screening. To explore the prevalence and rate of Gadolinium accumulation in different subgroups, we retrospectively analyzed MRIs of patients with von Hippel-Lindau disease (VHL) and Tuberous Sclerosis Complex (TSC).MethodsWe determined the prevalence and rate of accumulation in the dentate nucleus and globus pallidus on unenhanced T1-weighted MRI from VHL and TSC patients. We compared the signal intensities of these regions to the signal intensity of the pons. We evaluated the impact of number of MRIs, kidney function and liver function on Gadolinium accumulation.ResultsTwenty eight VHL patients and 24 TSC patients were included. The prevalence of accumulation in the dentate nucleus and globus pallidus increased linearly according to number of Gadolinium enhanced MRIs and was higher in the VHL group (100%). A significant linear correlation between number of MRIs and increased signal intensity was observed in the VHL group.ConclusionsGadolinium accumulation occurs in almost all patients undergoing contrast MRI screening after >5 MRIs. We advocate a screening protocol for patients with hereditary tumor syndromes that minimizes the Gadolinium dose. This can be accomplished by using a single administration to simultaneously screen for brain, spine and/or abdominal lesions, using an MRI protocol focused on either VHL- or TSC-specific lesions. Higher prevalence and rate of accumulation in VHL patients may be explained by the typical vascular leakage accompanying central nervous system hemangioblastomas.

Highlights

Patients with hereditary tumor syndromes undergo periodical magnetic resonance imaging (MRI) screening with Gadolinium contrast
We believe that the evaluation of kidney function is especially relevant in this setting, because some von Hippel-Lindau disease (VHL) and Tuberous Sclerosis Complex (TSC) patients have lost some of their kidney function due to renal cell carcinoma and angiomyolipomas, respectively
Our results demonstrate that the accumulation of Gd occurs in almost all patients who are regularly screened with Gd enhanced MRIs, especially after more than 5 MRIs

Summary

Introduction

Patients with hereditary tumor syndromes undergo periodical magnetic resonance imaging (MRI) screening with Gadolinium contrast. To explore the prevalence and rate of Gadolinium accumulation in different subgroups, we retrospectively analyzed MRIs of patients with von Hippel-Lindau disease (VHL) and Tuberous Sclerosis Complex (TSC). Patients with hereditary tumor syndromes need to undergo periodical magnetic resonance imaging (MRI) screening, according to international guidelines [1, 2] these patients are repeatedly exposed to intravenous Gadolinium (Gd) based contrast agents (GBCAs). After the Food and Drug Administration published a Public Health Advisory (2006) on GBCAs and the associated risk of nephrogenic systemic sclerosis, the use of GBCAs has been globally reduced, especially in patients with severe renal impairment. Accumulation has been described for both linear and macrocyclic GBCAs [7]

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