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tufted hemangioma
MIM.607859
Wednesday 26 November 2003
tufted angioma
Definition: Angioblastoma is a rare, benign vascular tumour composed of undifferentiated mesenchymal cells with a tendency to form lumina. This entity was first described by Nakagawa in 1949 as angioblastoma, and Wilson Jones was the first to use the term "tufted angioma" in 1976. Tufted angiomas usually occur in infancy and spread slowly. (MIM.607859)
Image
tufted angioma
A vascular tumor of kaposiform hemangioendothelioma / tufted angioma spectrum in an infant. Note the characteristic podoplanin / D2-40 stain
Epidemiology
sporadic or familial (autosomal dominant with reduced penetrance)
usually occurs in children younger than 5 years
may occur in adults (also in pregnancy)
Localization
restricted to skin (neck, shoulders, or trunk)
Macroscopy
exophytic, red lesion
Microscopy
’cannonball’ distribution of capillary-sized vessels in the dermis
vessels may form rounded nodules (’tufts’)
lymphatic vessels present at the periphery of lesion
Associations
Crohn disease receiving infliximab in addition to long standing azathioprine (15677546)
immunosuppression
Evolution
slow, progressive growth, then stable
may regress
References
Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. Le Huu AR, Jokinen CH, Ruben BP, Mihm MC, Weiss SW, North PE, Dadras SS. Am J Surg Pathol. 2010 Nov;34(11):1563-73. PMID: 20975337
Eruptive tufted angiomas in a patient with Crohn’s disease. Al-Za’abi AM, Ghazarian D, Greenberg GR, Shaw JC. J Clin Pathol. 2005 Feb;58(2):214-6. PMID: 15677546