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Peripheral primitive neuroectodermal tumor

Disease definition

A rare, aggressive, malignant, neoplastic disease characterized by a usually ill-defined, solid, multilobulated mass, frequently having necrosis, located on any site of the body (except the central nervous system), composed of small, round, poorly differentiated cells, with or without Homer-Wright rosettes, showing varying degrees of neuroectodermal differentiation. Manifestations are variable depending on location, with osteolytic destruction being common when arising from bone.


Classification level: Disorder
  • Synonym(s):
    • PPNET
    • Peripheral PNET
    • Peripheral neuroepithelioma
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult, Childhood, Adolescent
  • ICD-10: C71.9
  • OMIM: 612219
  • UMLS: C0684337
  • MeSH: D018241
  • GARD: -
  • MedDRA: -

Detailed information

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Disease review articles

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