Periodic Synopsis
Infantile hemangiomas

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Introduction

Infantile hemangiomas (IHs), also known as “hemangiomas of infancy,” are common benign tumors of endothelial cells characterized by a unique pattern of rapid proliferation that occurs in the first months of life, followed by slow involution that may take years to complete. They reportedly occur in as many as 10% of children and are frequently brought to the attention of both pediatricians and dermatologists. Although most are ultimately of little significance, a portion have the potential to produce complications of concern.

Confusion often arises regarding the diagnosis and management of IH, in part because the term “hemangioma” has been applied indiscriminately to a variety of vascular anomalies with diverse biologic and pathologic features. Clinical observations and pathologic studies, however, have clearly distinguished IHs, which are dynamic, from other vascular tumors and from vascular malformations. Because of this distinction, the terms “infantile hemangioma” or “hemangioma of infancy” should be used to describe these lesions, and older terms such as “strawberry” or “capillary” hemangioma should be discouraged. During the past decade, there have been an increasing number of studies and publications on IH. Highlights include the identification of specific immunohistochemical markers shared by placental microvasculature that distinguish IH from other vascular tumors, the recognition that Kasabach-Merritt phenomenon is not a complication of IH but rather of other vascular tumors, the description of an IH-specific neurocutaneous syndrome (PHACE(S) syndrome; posterior fossa malformation, hemangioma, arterial anomalies, coarctation of the aorta, eye abnormalities, and sternal defects), and the recognition of specific IH patterns that help in risk-stratification.

This periodic synopsis is by no means a complete compendium, but attempts to highlight those articles viewed by the authors as being among the most relevant and important. As the scope of all vascular tumors is broad and varied, we have restricted our focus (apart from issues of differential diagnosis) to the subject of IHs.

Section snippets

Review articles

Bruckner AL, Frieden IJ. Hemangiomas of infancy. J Am Acad Dermatol 2003;48:477-93.

Gampper TJ, Morgan RF. Vascular anomalies: hemangiomas. Plast Reconstr Surg 2002;110:572-85.

These recent reviews (although written for different audiences) provide comprehensive overviews of the subject at hand. Both include discussions of hemangioma classification, natural history, and management options. Bruckner and Frieden also focus on “worrisome presentations,” those hemangiomas most likely to produce

Pathology/pathogenesis

IHs are primarily composed of endothelial cells but also contain pericytes, fibroblasts, interstitial cells, and mast cells. The pathophysiologic mechanisms leading to endothelial cell proliferation and involution are poorly understood, and the variety of articles on the subject reflects many competing theories.

Clinical patterns, including segmental hemangiomas

Chiller KG, Passaro D, Frieden IJ. Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex. Arch Dermatol 2002;138:1567-76.

Haggstrom AN, Lammer EJ, Schneider RA, Marcucio R, Frieden IJ. Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development. Pediatrics 2006;117:698-703.

Waner M, North PE, Scherer KA, Frieden IJ, Waner A, Mihm MC Jr. The nonrandom distribution of facial

Ulceration

Kim HJ, Colombo M, Frieden IJ. Ulcerated hemangiomas: clinical characteristics and response to therapy. J Am Acad Dermatol 2001;44:962-72.

Wananukul S, Chatproedprai S. Ulcerated hemangiomas: clinical features and management. J Med Assoc Thai 2002;85:1220-5.

Ulceration is the most common complication of hemangiomas, occurring in approximately 15% of cases. The articles by Kim, Colombo, and Frieden and Wananukul and Chatproedprai are relatively large retrospective case series (60 and 41,

Differential diagnosis

The diagnosis of IH is usually straightforward, but other vascular malformations and tumors, as well as soft tissue tumors must be considered.

Hand JL, Frieden IJ. Vascular birthmarks of infancy: resolving nosologic confusion. Am J Med Genet 2002;108:257-64.

The term “hemangioma” has been applied to a variety of disparate vascular lesions in the past. Hand and Frieden reviewed the use of the term “hemangioma” in common genetic texts and found agreement with the definition established by the

Imaging

Burrows PE, Laor T, Paltiel H, Robertson RL. Diagnostic imaging in the evaluation of vascular birthmarks. Dermatol Clin 1998;16:455-88.

Dubois J, Garel L. Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group. Pediatr Radiol 1999;29:879-93.

Dubois J, Garel L, David M, Powell J. Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. Am J Roentgenol 2002;178:1541-5.

Konez O, Burrows PE. Magnetic resonance of vascular

Management

The management of hemangiomas is an area of great controversy. Only one double-blind controlled study (of pulsed dye laser) has been performed for hemangioma treatment, and there are no medical treatments that have been approved by the Food and Drug Administration. These articles reflect both knowledge and uncertainty in hemangioma management.

Psychosocial impact

Tanner JL, Dechert MP, Frieden IJ. Growing up with a facial hemangioma; parent and child coping and adaptation. Pediatrics 1998;101:446-52.

Gleason T. Summer's strawberry. J Am Acad Dermatol 2004;51(Suppl):S53-4.

One of the most important aspects of hemangiomas is their potential for either temporary or permanent disfigurement. Since more than half are on the head and neck, even those which do not cause specific medical complications can be truly life-altering. Only a limited number of studies

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Cited by (49)

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    2013, International Journal of Pediatric Otorhinolaryngology
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    An IH has the characteristic to proliferate rapidly in the first year of life. Most IHs proceed to resolution over a period of months to years without complications, not requiring any medical intervention [1]. The clinical significance of IHs varies widely and is often linked to their location, size and type.

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    Infantile hemangioma, a benign tumor of vascular endothelial cells (ECs), is the most common type of childhood tumor (Bruckner and Frieden, 2006; Mulliken and Glowacki, 1982).

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This report reflects the best data available at the time the report was prepared, but caution should be exercised in interpreting the data; the results of future studies may require alteration of the conclusions or recommendations set forth in this report.

Funding sources: None.

Conflicts of interest: None identified.

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