Non-involuting congenital hemangioma

Non-involuting congenital hemangioma (NICH) is a rare vascular tumor that is fully formed at birth and, unlike the more common infantile hemangiomas, does not undergo spontaneous regression or involution over time. NICH belongs to the group of congenital hemangiomas, which also includes rapidly involuting congenital hemangioma (RICH) and partially involuting congenital hemangioma (PICH). These lesions are distinct from infantile hemangiomas both clinically and histologically, and they are notably GLUT1-negative on immunohistochemistry, which helps differentiate them from infantile hemangiomas. NICH typically presents as a solitary, well-circumscribed, raised or flat vascular plaque or nodule on the skin. The lesion is often pink, violaceous, or bluish in color, sometimes with a pale rim or prominent overlying telangiectasias. It can occur anywhere on the body, including the head, trunk, and extremities. Because NICH persists indefinitely without regression, it may cause cosmetic concerns, and in some cases, it can be associated with local warmth, increased blood flow, or mild coagulopathy. The lesion primarily affects the skin and subcutaneous tissues, though deeper soft tissue involvement can occasionally occur. Treatment of NICH is primarily surgical excision when the lesion causes functional impairment, cosmetic disfigurement, or complications such as pain or ulceration. Unlike infantile hemangiomas, NICH does not respond to beta-blocker therapy (such as propranolol) or corticosteroids. In cases where surgery is not feasible or desired, observation and monitoring may be appropriate, as the lesions are generally benign. Embolization may be considered in select cases with significant arteriovenous shunting. Long-term prognosis is generally favorable, as malignant transformation has not been reported.

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