Congenital tufted angioma: Case report and review of the literature

Dermatología, Hospital General Universitario De Alicante, Alicante, Spain.
Dermatology online journal 05/2010; 16(5):2.
Source: PubMed


Tufted angiomas (TA) are rare benign vascular tumors of unknown pathogenesis. Most appear during childhood; approximately 25 percent are congenital and 50 percent appear in the first year of life. According to the literature, TA that are present at birth or in the first year of life have a greater tendency to spontaneously regress than do those that appear later in life. Their clinical presentation is non-specific and characterized by bluish-erythmatous plaques or nodules. The differential diagnosis includes infantile hemangiomas, congenital hemangiomas, kaposiform hemangioendothelioma and vascular malformations. Tufted angiomas have a characteristic histology consisting of a proliferation of endothelial cells forming lobules with the typical "shotgun" distribution. We report a case of congenital TA and review the cases of congenital TA described to date in the literature in order to highlight the different characteristics of congenital and acquired TA.

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    • "However, as the name suggests, the lesion was seen as cannon ball like, small circumscribed angiomatous tufts and nodules in the dermis and subcutaneous tissue with characteristic lymphangioma-like vessels [3]. 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]. It commonly presents as a macule, papule or plaque over the upper trunk, neck and proximal part of the limbs [6], however involvement of other locations like face, oral mucosa and lip [5] is also known. "
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    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 Slides The virtual slide(s) for this article can be found here:
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    ABSTRACT: Tufted angioma (TA), an uncommon benign vascular tumor, has a variable clinical presentation, and histopathologic findings are a key component of diagnosis. The presence of lymphatic vessels suggestive of lymphangioma can occasionally be the predominant finding and cause diagnostic confusion. Nine biopsies from 7 cases of TA were studied to assess the frequency and distribution of lymphangioma-like areas. Specimens were also stained with D2-40, VEGF-A, GLUT-1, and HHV-8. In one biopsy, lymphangioma-like vessels were the main finding. In all other cases of TA, lymphatics were present in the stroma but were often overshadowed by tufts of capillaries. D2-40 highlighted the stromal lymphatics and partially stained the capillaries within tufts. VEGF-A showed diffuse nonspecific staining of epidermis and endothelial cells in all specimens. GLUT1 and HHV-8 staining were uniformly negative in all 9 specimens. Accurate diagnosis of TA has important clinical implications given its occasional association with Kasabach-Merritt phenomenon, and the presence of lymphangioma-like vessels in biopsies of vascular lesions is entirely compatible with TA.
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    Indian journal of dermatology, venereology and leprology 03/2012; 78(2):231. DOI:10.4103/0378-6323.93667 · 1.39 Impact Factor
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