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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.

ORPHA:370348

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: -

  A summary on this disease is available in Deutsch (2019) Español (2019) Français (2019) Italiano (2019) Nederlands (2019)

Detailed information

General public

Guidelines

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.