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MRI features of benign peripheral nerve sheath tumors: how do sporadic and syndromic tumors differ?

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Abstract

Objectives

To compare MRI features of sporadic and neurofibromatosis syndrome–related localized schwannomas and neurofibromas.

Methods

In this retrospective study, our pathology database was searched for “neurofibroma” or “schwannoma” from 2014 to 2019. Exclusion criteria were lack of available MRI and intradural or plexiform tumors. Qualitative and quantitative anatomic (location, size, relationship to nerve, signal, muscle denervation) and functional (arterial enhancement, apparent diffusion-weighted coefficient) MRI features of sporadic and syndrome-related tumors were compared. Statistical significance was assumed for p < 0.05.

Results

A total of 80 patients with 64 schwannomas (sporadic: 42 (65.6%) v. syndrome-related: 22 (34.4%)) and 19 neurofibromas (sporadic: 7 (36.8%) v. syndrome-related: 12 (41.7%)) were included. Only signal heterogeneity (T2W p=0.001, post-contrast p=0.03) and a diffused-weighted imaging target sign (p=0.04) were more frequent with schwannomas than neurofibromas. Sporadic schwannomas were similar in size to syndrome-related schwannomas (2.9±1.2cm vs. 3.7±3.2 cm, p = 0.6), but with greater heterogeneity (T2W p = 0.02, post-contrast p = 0.01). Sporadic neurofibromas were larger (4.6±1.5cm vs. 3.4±2.4 cm, p = 0.03) than syndrome-related neurofibromas, also with greater heterogeneity (T2W p=0.03, post-contrast p=0.04). Additional tumors along an affected nerve were only observed with syndrome-related tumors). There was no difference in apparent diffusion coefficient values or presence of early perfusion between sporadic and syndrome-related tumors (p > 0.05).

Conclusions

Although syndrome-related and sporadic schwannomas and neurofibromas overlap in their anatomic, diffusion and perfusion features, signal heterogeneity and presence of multiple lesions along a nerve are differentiating characteristics of syndrome-related tumors.

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Data Availability

The data that support the findings of this study are available from the corresponding author, P.D., upon reasonable request.

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Authors and Affiliations

  1. The Russell H. Morgan Department of Radiology & Radiological Science, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD, 21287, USA

    Patrick Debs, Rodrigo Luna, Laura M. Fayad & Shivani Ahlawat

  2. Division of Orthopaedic Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Laura M. Fayad

Authors
  1. Patrick Debs
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  2. Rodrigo Luna
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  3. Laura M. Fayad
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  4. Shivani Ahlawat
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Correspondence to Patrick Debs.

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Debs, P., Luna, R., Fayad, L.M. et al. MRI features of benign peripheral nerve sheath tumors: how do sporadic and syndromic tumors differ?. Skeletal Radiol 53, 709–723 (2024). https://doi.org/10.1007/s00256-023-04479-1

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  • DOI: https://doi.org/10.1007/s00256-023-04479-1

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