Tufted angioma (Angioblastoma) of eyelid in adults-report of two cases

Ruchi Mittal,Devjyoti Tripathy

doi:10.1186/1746-1596-8-153

Abstract

Tufted angioma, first recognized in Japanese literature as “Angioblastoma of Nagakawa”, is a rare benign vascular tumour with a variable clinical presentation. It commonly manifests as a macule, papule or nodule in infancy or childhood in the region of the upper trunk and neck. Here in we report two cases of this rare progressive angioma as lesions of the eyelid in adults. Tufted angioma has a classical “cannon ball” like appearance of vascular tufts on histopathology. Immunohistochemical staining with actin highlights the spindly stromal cells surrounding the capillaries. Complete physical examination and haematological work up is recommended in patients with tufted angioma to exclude rare association of port wine stain and Kasabach-Merritt syndrome with this rare entity. To the best of our knowledge, our cases illustrate the first case report of tufted angioma presenting as an eyelid lesion.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1230909536950947.

Highlights

Tufted angioma, first recognized in Japanese literature as “Angioblastoma of Nagakawa”, is a benign progressive angioma, with a variable clinical presentation [1]
Wilson-Jones and Orkin [2] coined the term “tufted angioma” for this unusual pattern of angiomatous proliferation which was found to have some morphological similarities with strawberry nevi
Tufted angioma (TA) can be congenital or acquired, commonly presents in infancy or early childhood, can be present at birth in approximately 25% of cases [4], and few cases of TA have been reported in adults [5]

Summary

Background

First recognized in Japanese literature as “Angioblastoma of Nagakawa”, is a benign progressive angioma, with a variable clinical presentation [1]. Clinical examination revealed a right lower eyelid soft tissue mass not fixed to the underlying tarsus. It was non-tender and spongy to firm on palpation. Histopathology sections from case 1 showed multiple fragments of stromal tissue of eyelid with adnexal structures, striated muscle bundles of orbicularis oculi (Figure 1) and relatively circumscribed ovoid foci of closely set capillaries scattered throughout the stroma (Figures 1 and 2). Bundles of orbicularis oculi and closely set relatively circumscribed ovoid to round angiomatous aggregates These angiomatous aggregates were composed of blood less capillaries with slit like or mildly dilated lumina, lined by plump bland endothelial cells (Figure 3). No mitotic figure was identified in the sections studied Immunohistochemical staining in both cases showed similar findings. Both cases have not had a recurrence in over 12 months of follow up

Findings

Discussion

Conclusions