Peripheral primitive neuroectodermal tumor
Synonyms: PPNET | Peripheral PNET | Peripheral neuroepithelioma
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.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version September 2023.
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Peripheral primitive neuroectodermal tumor?
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