Noninvoluting congenital hemangioma: A rare cutaneous vascular anomaly

The authors studied a rare, congenital, cutaneous vascular anomaly that gro ws proportionately with the child and does not regress. A total of 53 patie nts were compiled from three vascular anomaly centers. These patients' lesi ons were analyzed for presentation, physical findings, radiologic and histo pathologic characteristics, natural history, and outcome after resection. The lesions occurred slightly more often in male patients, always appeared alone, and were located (in order of frequency) in the head/neck region, ex tremities, and trunk. The) were round-to-ovoid in shape, were plaque-like o r bossed, occurred in variable shades of pink to purple, and had an average diameter of 5 cm, The overlying skin was frequently punctuated by coarse t elangiectasia, often with central or peripheral pallor. The lesions were wa rm on palpation: fast-flow was fur ther documented by Doppler ultrasonograp hy. Magnetic resonance imaging and angiographic findings were similar to th ose of common hemangioma of infancy, All lesions were easily excised withou t recurrence. Histologic examination revealed lobular collections of small, thin-walled v essels with a large, often stellate, central vessel. Interlobular areas con tained predominantly dilated, often dysplastic veins: arteries were also in creased in number. Small artel ies were observed "shunting" directly into l obular vessels or into abnormal extralobular veins, "Hobnailed" endothelial cells lined the small intralobular vessels. Mast cells were increased. Tes ts for glucose transporter-1, a recently reported reliable marker for commo n hemangioma of infancy, were negative in all 26 specimens examined. In conclusion, the authors think these clinicopathologic and radiologic fea tures define a rare vascular lesion for which the term "noninvoluting conge nital hemangioma" is proposed. These lesions of intrauterine onset may be a variant of common hemangioma of infancy or another hemangiomatous entity w ith persistent fast-now.