Aim: The aim of this study is to have a further insight into this vascular tumor both clinically and histopathologically.

Methods: We examined 6 cases of acral arteriovenous tumor. Histochemically Masson trichrom, elastic van Gieson and immunohistochemically Desmin, Factor VIII, S100 and Mast cell tryptase were applied to the paraffin sections to evaluate the content of the vessels and mast cell distribution within the lesion.

Results: Vessels had smooth muscle and laminae elastica in most of the lesions. Arteriovenous anastomosis and glomus cells were not detected in any of the lesions. Mast cell count in the lesion was 215.17+/-42.2 whereas in control cases it was 89.16+/- 28.44. (p<0.001)

Conclusions: We suggest that acral arteriovenous tumor is an arterial and venous originated neoplasm, not an embryonic remnant or an aneurysm.

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