Malignant peripheral nerve sheath tumours (MPNST)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Malignant peripheral nerve sheath tumours (MPNST). Age 20-35 years. Fully referenced neurology checklist.

Risk factors

  • Age 20-35 years
  • Pre-existing plexiform neurofibromas: 18% transform
  • Previous radiotherapy
  • Previous personal or family history of cancer
  • Optic pathway glioma
  • Whole gene deletion
  • Multiple subcutaneous neurofibromas
  • Neurofibromatous neuropathy

Indicators of transformation to MPNST

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Investigations

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Treatment

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Outcome

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References

  1. Ferner RE, Huson SM, Thomas N, et al. Guidelines for the diagnosis and management of individuals with neurofibromatosis 1. J Med Genet 2007; 44:81-88. 
  2. Tucker T, Friedman JM, Friedrich RE, Wenzel R, Funsterer C, Mautner VF. Longitudinal study of neurofibromatosis 1 associated plexiform neurofibromas. J Med Genet 2009; 46:81-85.
  3. Pannu AK, Sharma N. Neurofibromatosis type 1 and disseminated malignant peripheral nerve sheath tumor. QJM 2017; 110:583-584. 
  4. James AW, Shurell E, Singh A, Dry SM, Eilber FC. Malignant peripheral nerve sheath tumor. Surg Oncol Clin N Am 2016; 25:789-802. 
  5. Schaefer IM, Fletcher CD, Hornick JL. Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics. Mod Pathol 2016; 29:4-13. 
  6. And 1 more. Subscribe to see the full list

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