This 2-year-old boy was born with a vascular nodule on the right clavicular area. Contrary to expectations, this asymptomatic lesion has continued to slowly increase in size proportion to the growth of the patient. Congenital hemangiomas differ from common hemangiomas of infancy (HOI) by developing in utero and presenting as fully grown tumors at birth. In addition, they lack a female preponderance. Congenital hemangiomas can be subdivided into rapidly involuting congenital hemangiomas (RICH), of which 50 percent involute prior to 7 months, and noninvoluting congenital hemangiomas (NICH). As opposed to common HOI, which often involute by 5 to 9 years of age, NICH never disappear.
Enjorlas et. al. studied the features of NICH in 53 patients from 3 vascular anomaly centers. All lesions were single and had an average diameter of 5cm. Most lesions were on the head and neck (43 percent ), followed by the limbs (38 percent ) and the trunk (19 percent ). Characteristic features included coarse overlying telangiectasias, pale halos at the rims, and intermingled areas of pallor. All lesions felt warm and were fast-flow by Doppler. MRI and angiography showed features similar to common HOI. Arteriography did not indicate early venous drainage. Therefore, these lesions were not true AV fistulas or AV malformations, entities which are also noninvoluting and were erroneously suspected to be involved in NICH. Histologically, NICH differed from common HOI by having larger and more irregular intralobular vessels, higher cellularity, multiple microscopic AV fistulas, and lack of staining for the glucose transporter-1. Although a few lesions temporarily improved soon after birth, most remained unchanged or slightly expanded as the child grew. 28 of the 53 lesions were resected, 7 of which had prior arterial embolization. However, all resections were without excessive bleeding, post-operative complications, or recurrence. Embolization alone was not evaluated.
There is still debate as to whether NICH is a true hemangioma or a vascular malformation with a proliferative component. However, it appears that NICH can be successfully treated by surgical excision without the need for prior embolization.
References:
1. Chiaverini C, Kurzenne JY, Rogopoulos A, Ortonne JP, Lacour JP. Noninvoluting congenital hemangioma: 2 cases. Ann Dermatol Venereol. 2002;129:735-7.
2. Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE. Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly.
Plast Reconstr Surg. 2001;107:1647-54.
Description
40 x 55 mm bluish patch on the right clavicular area with an overlying red telangiectatic vascular mass.
