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Comprehensive anatomical and functional imaging in patients with type I neurofibromatosis using simultaneous FDG-PET/MRI

  • Christian Philipp Reinert
  • Martin Ulrich Schuhmann
  • Benjamin Bender
  • Isabel Gugel
  • Christian la Fougère
  • Jürgen Schäfer
  • Sergios Gatidis
Original Article

Abstract

Purpose

To demonstrate the clinical use of FDG-PET/MRI for monitoring enlargement and metabolism of plexiform neurofibromas (PNF) in patients with neurofibromatosis type 1 (NF1), in whom the development of a malignant peripheral nerve sheath tumor (MPNST) is often a life limiting event.

Methods

NF1 patients who underwent a simultaneous FDG-PET/MRI examination in our institution from September 2012 to February 2018 were included. Indication was suspicion of malignant transformation of a PNF to MPNST. A maximum of six peripheral nerve lesions per patient were defined as targets. Standardized uptake values (SUV) and apparent diffusion coefficients (ADC) were measured. The presence of target sign and contrast-medium enhancement was visually recorded. Growth rates were estimated comparing prior or follow-up examinations and correlated with FDG uptake and ADC values. The presence of CNS lesions in cerebral T2 weighted images was recorded.

Results

In 28 NF1 patients a total number of 83 peripheral nerve tumors, 75 benign PNFs and eight MPNSTs, were selected as target lesions. The SUVs of MPNSTs were significantly higher than the SUVs of PNF (3.84 ± 3.98 [SUVmean MPNSTs] vs. 1.85 ± 1.03 [SUVmean PNF], P < .01). Similarly, lesion SUVmean-to-liver SUVmean ratios significantly differed between MPNSTs and PNF (3.20 ± 2.70 [MPNSTs] vs. 1.23 ± 0.61 [PNF]; P < .01). For differentiation between still benign PNF and MPNSTs, we defined SUVmax ≥ 2.78 as a significant cut-off value. Growth rate of PNF correlated significantly positively with SUVmean (rs = .41; P = .003). MRI parameters like ADCmean (1.87 ± 0.24 × 10−3 mm2/s [PNF] vs. 1.76 ± 0.11 × 10−3 mm2/s [MPNSTs]; P > .05], contrast medium enhancement (P = .50) and target sign (P = .86) did not differ between groups.

Conclusion

Simultaneous FDG-PET/MRI is a comprehensive imaging modality for monitoring PNF in NF1 patients. The combined acquisition of both morphologic information in MRI and metabolic information in PET enables the correlation of lesion growth rates with metabolic activity and to define SUV thresholds of significance to identify malignant transformation, which is of utmost clinical significance.

Keywords

PET/MRI Neurofibromatosis type 1 Long-term assessment Malignant peripheral nerve sheath tumor 

Notes

Compliance with ethical standards

Conflict of interest

Benjamin Bender received travel support by Bayer Vital and consultancy fees from Medtronic.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

This retrospective study was approved by our local institutional ethical review board and informed consent was waived (Project Number: 345/2018BO).

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Christian Philipp Reinert
    • 1
  • Martin Ulrich Schuhmann
    • 2
    • 3
  • Benjamin Bender
    • 4
  • Isabel Gugel
    • 2
    • 3
  • Christian la Fougère
    • 5
  • Jürgen Schäfer
    • 1
  • Sergios Gatidis
    • 1
  1. 1.Department of Diagnostic and Interventional RadiologyUniversity Hospital TuebingenTuebingenGermany
  2. 2.Department of NeurosurgeryUniversity Hospital TuebingenTuebingenGermany
  3. 3.Centre of Neurofibromatosis, Centre of Rare DiseasesUniversity Hospital TuebingenTuebingenGermany
  4. 4.Department of Diagnostic and Interventional NeuroradiologyUniversity Hospital TuebingenTuebingenGermany
  5. 5.Department of Nuclear Medicine and Clinical Molecular ImagingUniversity Hospital TuebingenTuebingenGermany

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