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Malignant peripheral nerve sheath tumor

Disease definition

Malignant peripheral nerve sheath tumor (MPNST) is a rare and often aggressive soft tissue sarcoma occurring in a wide range of anatomical sites.

ORPHA:3148

  • Synonym(s):
    • MPNST
    • Malignant neurilemmoma
    • Malignant neurofibroma
    • Malignant schwannoma
    • Neurofibrosarcoma
    • Neurogenic sarcoma
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Childhood, Adolescent, Adult, Elderly
  • ICD-10: C47.9
  • OMIM: -
  • UMLS: C0206729  C0751690
  • MeSH: -
  • GARD: 10872  8211
  • MedDRA: 10029236

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