Maximizing knowledge about infantile hemangioma with minimal or arrested growth
Feb. 3, 2017
Knowledge about hemangiomas is proliferating at a rate the compares with the first few days of an infantile hemangioma (IH) itself. Over the past decade a “new” lesion is coming into focus — the infantile hemangioma with minimal or arrested growth (IH-MAG).
Although previously recognized by a variety of names such as “plaque telangiectatic hemangiomas”, in 2008 Corella et al defined IH-MAGs as true infantile hemangiomas, recognized as precursor lesions that never undergo a growth phase or that undergo minimal growth. The authors described 4 patients with macular lesions that resembled precursor lesions of hemangiomas. The histology of all lesions demonstrated superficial ectatic vessels that reacted with anti-glucose transporter-1 (GLUT-1) antibodies, confirming that these are IHs (1). Even though these lesions may technically be congenital, they are distinct from the classical congenital hemangiomas (RICH – rapidly involuting or NICH – noninvoluting), which are GLUT-1 negative.
In a series of 47 patients with IH-MAGs, defined as IHs with a proliferative component < 25% of the total surface area, Suh and Freiden found that the lesions most commonly presented as fine or coarse telangiectatic patches. Proliferation was present in 30% (14 of 47 IH-MAGs), usually as small papules at the periphery. Sixty-eight percent (32 of 47 IH-MAGs) of them were present on the lower body. Seventeen patients had classic infantile hemangiomas at another body site. Comparison of distribution of sites of IH-MAGs showed a 26-fold (95% confidence interval, 1.9-351.5; P = .01) likelihood of having IH-MAGs on the lower body compared with classic infantile hemangiomas. The authors noted that despite reports associating IH-MAGs with the PELVIS syndrome (perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag) and the SACRAL syndrome (spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localization), these associations are very rare. Only one patient in their series of 47 patients demonstrated spinal dysraphism on MRI (2).
The clinical variant of IH-MAG, labeled RIH-MAG (reticular infantile hemangioma with minimal or arrested growth), is manifested by macular lesions with a “network and blotchy” appearance that can be mistaken for a capillary malformation/port-wine stain. Bessis et al reported 7 cases with attendant lipoatrophy, with one case displaying the PELVIS syndrome (3).
Ma et al reviewed 9 cases of IH-MAG. All patients had serial photographs demonstrating resolution of the birthmark over time. Five of these cases had a skin biopsy performed, all of which confirmed GLUT-1 positivity. The authors concluded that IH-MAG is a unique clinical subset of hemangioma for which close observation is the preferred treatment. When in doubt, they suggested performing a biopsy for histology and GLUT-1 status to confirm the diagnosis before embarking on unnecessary laser treatment or medical interventions (4).
I am speculating, but I surmise that either propranolol or topical timolol would have some benefit in uncomplicated cases of IH-MAG, even though the prognosis is excellent. Their use would depend on parental anxiety and cosmetic concerns.
The $64,000 question of course is why some IHs progress and IH-MAGs do not. (The quiz show $64,000 question was first broadcast in 1955; with inflation it should technically be the $567,040 question.) The leading hypothesis focuses on a reaction to tissue hypoxia that induces the proliferation of endothelial progenitors, with subsequent vasculogenesis via mediators such as VEGF or hypoxia-inducible factor-1-alpha (3). In the future, a genuine understanding of the pathomechanism of why some infantile hemangiomas fail develop should allow for refinement of beta-blocker therapy.
1. Corella F, et al. Abortive or minimal-growth hemangiomas: Immunohistochemical evidence that they represent true infantile hemangiomas. J Am Acad Dermatol 2008; 58: 685-90.
2. Suh KY, Freiden IJ. Infantile hemangiomas with minimal or arrested growth: A retrospective case series. Arch Dermatol 2010; 146: 971-6
3. Bessis D, et al. Reticular infantile hemangiomas with minimal or arrested growth associated with lipoatrophy. J Am Acad Dermatol 2105; 72: 828-33.
4. Ma EH, et al. Infantile hemangioma with minimal or arrested growth: Further observations on clinical and histopathologic findings of this unique but underrecognized entity. Pediatr Dermatol 2017; 34: 64-71.
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