Hemangiomas

Published on 18/03/2015 by admin

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Last modified 18/03/2015

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Hemangiomas

Omar Pacha and Adelaide A. Hebert

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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A hemangioma is a benign neoplastic proliferation of endothelial cells and is the most common soft tissue tumor of infancy. The incidence of hemangiomas in a general newborn nursery is between 1% and 2.6%, but may be as high as 10% in the Caucasian population. Hemangiomas occur four times more frequently in female infants with a predilection for premature infants.

Management strategy

Approximately 55% of hemangiomas present at birth, with the remainder arising in the first weeks of life. Initially, mature cutaneous hemangiomas grow rapidly for the first 3 to 9 months. After this characteristic proliferative phase, the lesions typically cease growing at 18 months of age, and subsequent spontaneous involution is the rule. Most will involute slowly over a period of 2 to 6 years, usually completing the process by age of 7–10 years. About half of children with hemangiomas will have normal skin after involution, but the rest may have residual changes, including telangiectasias, atrophy, fibrofatty residuum, and scarring. Although for uncomplicated hemangiomas treatment typically consisted of active non-intervention, the advent of use of oral and systemic steroids and/or beta-blocking agents changed the therapeutic paradigm for these vascular tumors.

Differentiating benign, common hemangiomas from other vascular anomalies is essential as the pathophysiology, treatment modalities, and prognoses are significantly different. Vascular malformations and tumors such as the kaposiform hemangioendothelioma (KE) and tufted angioma (TA) differ from hemangioma in both clinical and histological appearance as well as growth rate and involutional tendencies. In particular, KE and TA are associated with the Kasabach–Merritt syndrome and its accompanying coagulopathy, whereas common hemangiomas are not. The majority of common hemangiomas occur as solitary lesions, but organ system involvement or, rarely, syndromes such as diffuse neonatal hemangiomatosis and the PHACES syndrome (posterior fossa malformation, hemangiomas, cardiac anomalies, eye abnormalities, and sternal cleft/supraumbilical raphe) can also occur.

Although the natural course of hemangiomas is self-limited, treatment is probably indicated for approximately 25%, including the 5% that ulcerate and the 20% that may compress, obstruct, or distort vital structures, such as the larynx, eyes, ears, and nose. Medical management of low-risk hemangiomas is generally centered on the administration of topical and systemic beta-blockers, corticosteroids, either topically or intralesionally. One group of investigators used clobetasol propionate cream 0.05% to treat periocular hemangiomas. Other less frequently used management options include cryosurgery, surgical excision, and laser therapy.

Topical and systemic beta-blockers are quickly becoming the preferred first-line therapy in hemangiomas that will require treatment. Starting in 2008, publication of successful treatment of large morbid hemangiomas with propranolol was described. Since that time, other iterations of beta-blocker therapy such as topical timolol have also been used. Current available recommendations for oral propranolol range from 0.5 mg/kg/day to 2 mg/kg/day divided into two to three daily doses. Timolol, available as solution or gel, has reported success in treating periocular and facial infantile hemangiomas with twice daily topical application.

Systemic corticosteroids may be added for larger, deforming, or life-threatening lesions, and are usually indicated during the growth phase. Prednisone or prednisolone can be given at doses from 2–4 mg/kg daily from 2 to 6 months, and then gradually tapered over several months. Stopping treatment before adequate therapeutic response may result in rebound growth. Approximately one-third of patients will show an accelerated rate of involution, but another one-third may have no response to this treatment modality. Reported risks include hypothalamopituitary–adrenal axis suppression, growth delays, pseudotumor cerebri, infections, and avascular bone necrosis. Surgical excision, laser treatment (especially flashlamp-pumped pulsed-dye laser), and cryosurgery, either alone or in combination with corticosteroids, may also be employed in certain cases.

For endangering or life-threatening lesions refractory to systemic corticosteroids, interferon-α2a or -α2b may be used. The typical delivery route is subcutaneously at a dose of 3 million units/m2 daily for 6 to 12 months. Side effects include transient neutropenia, fever, elevated liver enzymes, and flu-like symptoms. In addition, there are rare reports of neurotoxicity, specifically spastic diplegia, which may develop in 5–10% of patients. For the exceptional recalcitrant hemangioma, other treatments include cyclophosphamide, vincristine, bleomycin, propranolol, and embolization.

Specific investigations

The diagnosis of hemangioma is usually made clinically, and the above investigations may only be needed in atypical cases to monitor the progress of treatment, establish the extent of the vascular lesion, or screen for other complications.

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