Peripheral primitive neuroectodermal tumor

Orpha code: 370348OMIM code: 612219

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.

Disease data
Classification

Disease

Synonyms
PPNET
Obwodowy PNET
PPNET
Peripheral PNET
Peripheral neuroepithelioma
ORPHA code
370348
OMIM code
612219
ICD10 code
C71.9
ICD11 code
XH6P76

No additional description.

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