Hemangioma

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A hemangioma is a congenital[1] benign tumour or vascular malformation[2] of endothelial cells (the cells that line blood vessels).

In infancy, it is the most common tumor.[3]

Before considering the hemangioma it is important to understand that there have been recent changes in the terminology used to define vascular anomalies (abnormal lumps made up of blood vessels). The term hemangioma was originally used to describe any vascular tumour both present around birth or appearing later in life. Mulliken et al. separated these conditions into a family of self-involuting tumours (growing lesions that eventually disappear) from the family of malformations (enlarged or abnormal vessels present at birth and essentially permanent). The importance of this separation is that it allows us to differentiate early in life between lesions that will resolve versus those that are permanent. Examples of permanent malformations include Port-wine stains (capillary vascular malformation) and masses of abnormal swollen veins (venous malformations).[4] Unfortunately many textbooks and dictionaries are not up to date, creating great confusion.

Hemangiomas are connected to the circulatory system and filled with blood. The appearance depends on location. If they are on the surface of the skin they look like a ripe strawberry, if they are just under the skin they present as a bluish swelling. Sometimes they grow in internal organs such as the liver or larynx. In most cases, hemangiomas will disappear over time. They are formed either during gestation or most commonly they are not present at birth but appear during the first few weeks of life. They are often misdiagnosed, initially, as a scratch or bruise but the diagnosis becomes obvious with further growth. Typically at the earliest phase in a superficial lesion one will see a bluish red area with obvious blood vessels and surrounding pallor. Sometimes they present as a flat red or pink area. Hemangiomas are the most common childhood tumor, occurring in approximately ten percent of Caucasians, and are less prevalent in other races. Females are three to five times more likely to have hemangiomas than males. They are also more common in twin pregnancies. Approximately 80% are located on the face and neck, with the next most prevalent location being the liver. Although hemangiomas are benign, some serious complications can occur. Hemangiomas never develop as an adult but one misunderstanding is that all hemangiomas go away by the age of 10; very few do not.

The cause of hemangioma is currently unknown; however, several studies have suggested the importance of estrogen signaling in hemangioma proliferation. In 2007, a paper from the Stanford Children’s Surgical Laboratory revealed that localized soft tissue hypoxia coupled with increased circulating estrogen after birth may be the stimulus.[5] There is also a hypothesis presented by researchers at Harvard and the University of Arkansas that maternal placenta embolizes to the fetal dermis during gestation resulting in hemangiomagenesis,[6][7] yet Duke researchers conducted genetic analyses of small nucleotide polymorphisms in hemangioma tissue compared to the mother’s DNA that contradicted this notion.[8] More research is required in order to fully understand the explosive nature of hemangioma growth which will hopefully yield targeted therapeutics to treat its most complicated presentations

Hemangiomas may also be classified by location. Cerebellar hemangioma, for example, may cause polycythemia as a paraneoplastic syndrome.[9]

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