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PET imaging characteristics of neuromuscular choristoma and associated desmoid-type fibromatosis

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Abstract

Background

Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion characterized by abnormal presence of muscle within nerve. Associated desmoid-type fibromatosis (NMC-DTF) often develops. We report 18F-fluorodeoxyglucose positron emission tomography (FDG PET) characteristics of NMC and NMC-DTF and propose that increased FDG activity within NMCs may be associated with subclinical NMC-DTF or NMC-DTF “precursor” tissue.

Methods

Our institutional database was searched for all NMC cases. Inclusion criteria were 1) confirmed diagnosis of NMC with or without biopsy, and 2) available PET and MRI studies. PET data included SUVmax and SUVmean of NMCs, contralateral limb normal skeletal muscle and unaffected nerves, and SUVmax of NMC-DTF if present. SUV values were compared using paired t-test. A p value of < 0.05 was considered statistically significant.

Results

Our cohort consisted of 9 patients with NMC, 8 cases involving sciatic nerve and 1 of brachial plexus. On PET imaging, all NMC-affected nerve segments showed significantly higher FDG uptake (SUVmax/mean) compared to both contralateral normal nerve and normal skeletal muscle (all P < 0.05). Similar to sporadic DTF, NMC-DTF was highly FDG-avid (average SUVmax of 4.2). SUVmax in NMC with or without concurrent NMC-DTF did not differ (p = 0.76). Within NMC-affected nerve segment, FDG activity was relatively higher in areas with low T1/T2 MR signal.

Conclusion

All NMCs were more FDG avid compared to both normal skeletal muscle and contralateral unaffected nerve, arguing against the presence of heterotopic muscle in NMC as the source of FDG avidity. FDG avidity within NMC may reflect subclinical NMC-DTF or a precursor lesion, as NMC-DTF are highly FDG-avid, and the highest regions of FDG avidity in NMC occurred in regions with MR characteristics associated with NMC-DTF (i.e., lower T1/T2 signal). We believe that the integration of FDG PET with serial MR imaging in patient follow up will clarify its utility in both detection and surveillance of NMC-DTF.

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Data Availability

Data available upon reasonable request to the corresponding author.

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Authors and Affiliations

  1. Department of Neurologic Surgery, Mayo Clinic, Gonda 8-214, Rochester, MN, 55905, USA

    Tomas Marek & Robert J. Spinner

  2. Department of Radiology, University of Florida, Jacksonville, FL, USA

    Tomas Marek

  3. Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, Canada

    Jodi M. Carter

  4. Department of Neurologic Surgery, UC San Diego Health, San Diego, CA, USA

    Nikhil K. Murthy

  5. Department of Radiology, Mayo Clinic, Rochester, MN, USA

    Kimberly K. Amrami & Stephen M. Broski

Authors
  1. Tomas Marek
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  2. Robert J. Spinner
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  3. Jodi M. Carter
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  4. Nikhil K. Murthy
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  5. Kimberly K. Amrami
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  6. Stephen M. Broski
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Correspondence to Robert J. Spinner.

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This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Institutional Review Board of Mayo Clinic.

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Marek, T., Spinner, R.J., Carter, J.M. et al. PET imaging characteristics of neuromuscular choristoma and associated desmoid-type fibromatosis. Acta Neurochir 165, 1171–1177 (2023). https://doi.org/10.1007/s00701-023-05547-0

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