Peripheral primitive neuroectodermal tumor

Go to external page http://www.orpha.net/ORDO/Orphanet_370348


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.

Synonyms:

Peripheral PNET

PPNET

Peripheral neuroepithelioma

This is just here as a test because I lose it

Term information

database cross reference
expertlink
  • https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=370348
hasDbXref
  • ICD-10:C71.9
  • OMIM:612219
  • UMLS:C0684337
has age of onset
  • http://www.orpha.net/ORDO/Orphanet_409948
  • http://www.orpha.net/ORDO/Orphanet_409947
  • http://www.orpha.net/ORDO/Orphanet_409946
has inheritance
  • http://www.orpha.net/ORDO/Orphanet_409941
has point prevalence range
  • http://www.orpha.net/ORDO/Orphanet_409981
notation
  • ORPHA:370348
part of
  • http://www.orpha.net/ORDO/Orphanet_3394
  • http://www.orpha.net/ORDO/Orphanet_223727
present in
  • http://www.orpha.net/ORDO/Orphanet_409991