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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