Orphanet Rare Disease Ontology

Last uploaded: December 4, 2024
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Id http://www.orpha.net/ORDO/Orphanet_370348
http://www.orpha.net/ORDO/Orphanet_370348
Preferred Name

Peripheral primitive neuroectodermal tumor
Definitions
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 neuroepithelioma
PPNET
Peripheral PNET
Type http://www.w3.org/2002/07/owl#Class

All Properties

definition_citation

Orphanet
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.
label
Peripheral primitive neuroectodermal tumor
prefLabel
Peripheral primitive neuroectodermal tumor
hasDbXref
OMIM:612219
ICD-10:C71.9
MeSH:D018241
UMLS:C0684337
notation
ORPHA:370348
alternative_term
Peripheral neuroepithelioma
PPNET
Peripheral PNET
present_in
Worldwide AND has_point_prevalence_range : Unknown
part_of
prefixIRI
ORDO:Orphanet_370348
expertlink
subClassOf
type
has_inheritance
Not applicable
has_age_of_onset
Adolescent
Adult
Childhood
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