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Ангіоматоїдна фіброзна гістіоцитома

ORPHA:569164· ICD-10 D21.9· Angiomatoid fibrous histiocytoma

Визначення(English summary)

A rare soft tissue tumor characterized by a slow-growing, usually painless, subcutaneous nodule, predominantly located in the extremities, less frequently the trunk or head and neck region. Histopathologically, the lesion is well-circumscribed, lobulated, and composed of epitheloid, ovoid, or spindle cells arranged in a nodular and often syncytial pattern, with pseudoangiomatoid spaces and a peripheral fibrous pseudocapsule with a prominent lymphoplasmacytic cuff. The tumor is most common in the first two decades of life and usually follows an indolent course, although local recurrence may occur, while metastasis is rare.

Поширеність
Unknown
Вік початку
Adolescent, Adult, Childhood