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Retiform hemangioendothelioma

ORPHA:458763· ICD-10 D48.1

Definition

A rare vascular tumor characterized by a slowly growing lesion with predominant involvement of the skin and subcutaneous tissue of the distal extremities. Distinctive arborizing blood vessels lined by endothelial cells with characteristic hobnail morphology are a typical feature. Local recurrences are frequent unless wide local excision is performed, while metastasis is rare.

Prevalence
<1 / 1 000 000
Inheritance
Not applicable
Age of onset
Adolescent, Adult