Obwodowy prymitywny guz neuroektodermalny

Kod Orpha: 370348Kod OMIM: 612219

Definicja

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.

Dane
Klasyfikacja

Choroba

Synonimy
PPNET
Obwodowy PNET
PPNET
Peripheral PNET
Peripheral neuroepithelioma
Kod ORPHA
370348
Kod OMIM
612219
Kod ICD10
C71.9
Kod ICD11
XH6P76

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