Malignant peripheral nerve sheath tumours (MPNST)

Evidence-based neurology checklist on malignant peripheral nerve sheath tumours (mpnst): 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…

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

Investigations

Treatment

Outcome

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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