Identifying Hemangiomas vs Vascular Malformations

At Dermatology Week 2021, James Treat, MD, professor of clinical pediatrics and dermatology at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia, discussed the ins and outs of differentiating hemangiomas vs vascular malformations.

To start the session, Dr Treat stressed the importance of accurately defining both hemangiomas and vascular malformations. He pointed to the International Society for the Study of Vascular Anomalies (ISSVA) classifications for vascular anomalies. This resource, most recently revised in 2018, splits vascular anomalies into vascular tumors and vascular malformations, and the resource is available online at the ISSVA website.

With lesional definitions out of the way, Dr Treat moved onto discussing infantile hemangiomas. Typically, these lesions are not present at birth but begin to develop as a faint red or bruise-like patch at age 1 to 4 weeks that rapidly grows over the next 1 to 3 months until involution begins around 1 year. Treatments of choice are propranolol (FDA approved for as young as age 5 weeks) and timolol. Dr Treat said a few hemangiomas should be considered “risky” due to their location and should be referred to a specialist early, including:

• Beard distribution (risk of airway involvement)
• Periocular (vision risk)
• Lips (risk of obstruction of breathing or eating)
• Lumbosacral (risk of spinal cord anomalies)

Hemangiomas may also be risky due to ulceration and rapid growth, but dermatologists should be on the lookout for a segmental pattern, which is more commonly associated with underlying abnormalities.

A congenital hemangioma is present at birth, typically with an outer rim of vasoconstriction. Dr Treat indicated that it is important to feel these for a soft/squishy response, because sarcomas can appear similarly but are firm to touch.

After establishing what to look for in examining a hemangioma, Dr Treat shifted focus to capillary malformations. Nevus simplex is almost never a cause for concern (unless the patient also presents with macrocephaly, macroglossia, asymmetric growth, or limb anomalies) and will fade over time. Port wine stains (PWSs) can be associated with Sturge-Weber syndrome, particularly those with forehead involvement, whereas reticulated PWS is more associated with genetic syndromes and geometric PWS is more associated with underlying venous or venolympathic malformations.

Dr Treat also highlighted thumb-print oval capillary malformations, which can look like café au lait macules that are red-purple in color. It’s important to make sure patients who present with these lesions undergo genetic testing and early screening to monitor for arteriovenous malformations in the central nervous system.

PWS-like lesions that do not seem to be respecting the midline, explained Dr Treat, are tufted angiomas. These appear as red-purple patch or thin plaque, and it may be congenital. However, it is important to check laboratory results to rule out Kasabach-Merritt phenomenon (KMP), which may also be a concern for Kaposiform haemangioendotheliomas, and sirolimus is an effective therapy for KMP if necessary.

The last lesion to note, said Dr Treat, is actually not a hemangioma or vascular malformation at all. He explained infantile fibrosarcoma can be a mimic of hemangioma, but it is firm to touch and its growth does not respect tissue planes. If unsure about a presentation, Dr Treat recommended referral to an expert or performing a biopsy due to the sensitive nature of the lesion.

Reference
Treat J. Pediatric dermatology: hemangiomas and vascular malformations. Presented at: Dermatology Week 2021; September 16-19, 2021; virtual.