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Tumefactive brain parenchymal neurosarcoidosis

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Abstract

Background

Enhancing brain parenchymal disease, and especially tumefactive lesions, are an uncommon manifestation of neurosarcoidosis. Little is known about the clinical features of tumefactive lesions and their impact on management and outcomes, which this study aims to characterize.

Methods

Patients with pathologically-confirmed sarcoidosis were retrospectively reviewed and included if brain lesions were: (1) intraparenchymal, (2) larger than 1 cm in diameter, and (3) associated with edema and/or mass effect.

Results

Nine patients (9/214, 4.2%) were included. Median onset age was 37 years. Diagnosis was confirmed by brain parenchymal biopsies in 5 (55.6%). Median modified Rankin scale (mRS) score was 2 (range 1–4) at initial presentation. Common manifestations included headache (77.8%), cognitive dysfunction (66.7%), and seizures (44.4%). Sixteen lesions were present in 9 patients. The frontal lobe (31.3%) was most affected, followed by the subinsular region (12.5%), basal ganglia (12.5%%), cerebellum (12.5%), and pons (12.5%). MRI characteristics of the dominant lesions included spherical morphology (77.8%), perilesional edema (100.0%), mass effect (55.6%), well-demarcated borders (66.7%), and contrast enhancement (100.0%; 55.6% heterogeneous). Leptomeningitis was frequently present (77.8%). All required corticosteroid-sparing treatments, and most (55.6%) needed at least a third line of treatment (infliximab used in 44.4%). All patients relapsed (median 3 relapses, range 1–9). Median last mRS was 1.0 after median follow-up of 86 months, with significant residual deficits in 55.6%.

Conclusion

Tumefactive brain parenchymal lesions are uncommon, usually affect the supratentorial brain along with leptomeningitis, and are refractory to initial treatments with a high risk of relapse. Significant sequelae were encountered despite a favorable median last mRS.

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Data availability

Subject to ethics board approval, anonymized data will be provided to qualified investigators upon request.

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Funding

No funding was received for conducting this study.

Author information

Authors and Affiliations

  1. Department of Neurology, Emory University School of Medicine, 12 Executive Park Drive NE, Atlanta, GA, 30329, USA

    Gabriela A. Bou, Sally El Sammak, Julien J. Cavanagh & Spencer K. Hutto

  2. Department of Radiology, Emory University School of Medicine, Atlanta, GA, USA

    Ling-Chen Chien

Authors
  1. Gabriela A. Bou
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  2. Sally El Sammak
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  3. Ling-Chen Chien
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  4. Julien J. Cavanagh
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  5. Spencer K. Hutto
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Contributions

Conceptualization: SKH; Methodology: JJC, SKH; Formal analysis and investigation: GAB, SS, SKH; Figures – preparation: SS, LCC; Writing – original draft preparation: GAB, SKH; Writing – review and editing: SS, LCC, JJC, and SKH; Supervision: SKH.

Corresponding author

Correspondence to Spencer K. Hutto.

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Conflicts of interest

The authors have no competing interests to declare that are relevant to the content of this article.

Ethical approval

The Institutional Review Board of Emory University approved this retrospective cohort study and granted a complete waiver of consent in view of the retrospective nature of the study, and all procedures being performed were part of routine care. Subject to ethics board approval, anonymized data will be provided to qualified investigators upon request.

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Bou, G.A., El Sammak, S., Chien, LC. et al. Tumefactive brain parenchymal neurosarcoidosis. J Neurol 270, 4368–4376 (2023). https://doi.org/10.1007/s00415-023-11782-3

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  • DOI: https://doi.org/10.1007/s00415-023-11782-3

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