Neuroradiology

, Volume 45, Issue 9, pp 618–625 | Cite as

Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma

  • V. F. Mautner
  • R. E. Friedrich
  • A. von Deimling
  • C. Hagel
  • B. Korf
  • M. T. Knöfel
  • R. Wenzel
  • C. Fünsterer
Diagnostic Neuroradiology

Abstract

Plexiform neurofibroma (PNF) is a typical feature of neurofibromatosis 1 (NF1). About 10% of patients with NF1 develop malignant peripheral nerve-sheath tumours (MPNST), usually arising from PNF, and this is the major cause of poor survival. A better prognosis can be achieved if the tumours are diagnosed at an early stage. Our objective was to establish MRI criteria for MPNST and to test their usefulness in detecting early malignant change in PNF. MRI was performed on 50 patients with NF1 and nerve-sheath tumours, of whom seven had atypical pain, tumour growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic. On MRI all seven symptomatic patients had inhomogeneous lesions, due to necrosis and haemorrhage and patchy contrast enhancement. In one patient, the multiplicity of confluent tumours with inhomogeneous areas in addition to central lesions did not allow exclusion of malignancy. Only three of the 43 asymptomatic patients had comparable changes; the other 40 patients had tumours being of relatively homogeneous structure on T1- and T2-weighted images before and after contrast enhancement. All three asymptomatic patients with inhomogeneous lesions were shown to have MPNST.

Keywords

Neurofibromatosis type 1 Malignant peripheral nerve-sheath tumour Plexiform neurofibroma Magnetic resonance imaging 

Notes

Acknowledgements

This study was supported by Deutsche Krebshilfe Grant No. 70-2794-De 1 and the Hamburger Stiftung zur Förderung der Krebsbekämpfung No. 161.

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Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • V. F. Mautner
    • 1
  • R. E. Friedrich
    • 2
  • A. von Deimling
    • 3
  • C. Hagel
    • 4
  • B. Korf
    • 5
  • M. T. Knöfel
    • 6
  • R. Wenzel
    • 7
  • C. Fünsterer
    • 7
  1. 1.Department of NeurologyKlinikum Nord HamburgHamburgGermany
  2. 2.Department of Maxillofacial SurgeryUniversitätsklinikum EppendorfHamburgGermany
  3. 3.Department of NeuropathologyCharitéBerlinGermany
  4. 4.Department of NeuropathologyUniversitätsklinikum EppendorfHamburgGermany
  5. 5.Partners Center for Human GeneticsHarvard Institutes of MedicineBostonUSA
  6. 6.Department of SurgeryUniversitätsklinikum EppendorfHamburgGermany
  7. 7.MRI-Institute Hamburg OthmarschenHamburgGermany

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