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Периферична примітивна нейроектодермальна пухлина

ORPHA:370348· ICD-10 C71.9· Peripheral primitive neuroectodermal tumor

Визначення(English summary)

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.

Поширеність
Unknown
Успадкування
Not applicable
Вік початку
Adolescent, Adult, Childhood