Peripheral primitive neuroectodermal tumor

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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.


Peripheral PNET


Peripheral neuroepithelioma

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Term information

database cross reference
  • ICD-10:C71.9
  • OMIM:612219
  • UMLS:C0684337
has age of onset
has inheritance
has point prevalence range
  • ORPHA:370348
part of
present in