loading...| Preferred Name |
Peripheral primitive neuroectodermal tumor |
|
| Synonyms |
Peripheral neuroepithelioma |
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| 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. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_370348 |
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| alternative_term |
Peripheral neuroepithelioma PPNET Peripheral PNET |
|
| 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. |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=370348 |
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| has_age_of_onset |
Adolescent Adult Childhood |
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| has_inheritance |
Not applicable |
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| hasDbXref |
OMIM:612219 ICD-10:C71.9 MeSH:D018241 UMLS:C0684337 |
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| label |
Peripheral primitive neuroectodermal tumor |
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| notation |
ORPHA:370348 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_370348 |
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| prefLabel |
Peripheral primitive neuroectodermal tumor |
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| present_in |
Worldwide AND has_point_prevalence_range : Unknown |
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| treeView | ||
| subClassOf |