Angiomatoid fibrous histiocytoma

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Angiomatoid fibrous histiocytoma

Synonyms: AFH

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024

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