A
hemangioma of infancy is a
benignA benign tumor is a tumor that lacks all three of the malignant properties of a cancer. Thus, by definition, a benign tumor does not grow in an unlimited, aggressive manner, does not invade surrounding tissues, and does not metastasize...
self-involuting tumour of endothelial cells (the cells that line blood vessels). In most cases it appears during the first days or weeks of life and will have resolved at the latest by age 10. In infancy, it is the most common tumor.
The word "hemangioma" comes from the Greek word
hema- (Greek: αίμα) meaning "blood",
angeio (Greek: αγγείο) meaning "vessel" and the suffix
-oma (Greek: -ωμα) meaning "tumor".
Before considering the hemangioma, it is important to understand that there have been recent changes in the terminology used to define, describe and categorize vascular anomalies (abnormal lumps made up of blood vessels).
A
hemangioma of infancy is a
benignA benign tumor is a tumor that lacks all three of the malignant properties of a cancer. Thus, by definition, a benign tumor does not grow in an unlimited, aggressive manner, does not invade surrounding tissues, and does not metastasize...
self-involuting tumour of endothelial cells (the cells that line blood vessels). In most cases it appears during the first days or weeks of life and will have resolved at the latest by age 10. In infancy, it is the most common tumor.
The word "hemangioma" comes from the Greek word
hema- (Greek: αίμα) meaning "blood",
angeio (Greek: αγγείο) meaning "vessel" and the suffix
-oma (Greek: -ωμα) meaning "tumor".
Terminology
Before considering the hemangioma, it is important to understand that there have been recent changes in the terminology used to define, describe and categorize vascular anomalies (abnormal lumps made up of blood vessels). The term
hemangioma was originally used to describe any vascular tumour-like structure, whether it was present at or around birth or appeared later in life. Mulliken et al. categorized these conditions into two families; a family of self-involuting tumours (growing lesions that eventually disappear) and another family of malformations (enlarged or abnormal vessels present at birth and essentially permanent). The importance of this distinction is that it makes it possible for early-in-life differentiation between lesions that will resolve versus those that are permanent. Examples of permanent malformations include
Port-wine stainA port-wine stain or naevus flammeus is a vascular birthmark consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discolouration of the skin. They are so called for their colour, resembling that of port wine. It is part of the family of disorders...
s (capillary vascular malformation) and masses of abnormal swollen veins (venous malformations). Unfortunately many textbooks and dictionaries are not up to date, creating great confusion.
Presentation
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
gestationGestation is the carrying of an embryo or fetus inside a female viviparous animal. Mammals duringpregnancy can have one or more gestations at the same time ....
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.
Causes
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. 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, yet Duke researchers conducted genetic analyses of small nucleotide polymorphisms in hemangioma tissue compared to the mother's DNA that contradicted this notion. 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.
Complications
The vast majority of hemangiomas are not associated with complications. Hemangiomas may break down on the surface (ulcerate). If the ulceration is deep, significant bleeding may occur in rare occasions. Ulceration on the diaper area can be painful and problematic.
If a hemangioma develops in the larynx, breathing can be compromised. A hemangioma can grow and block one of the eyes, causing an
occlusion amblyopiaAmblyopia, otherwise known as lazy eye, is a disorder of the visual system that is characterized by poor or indistinct vision in an eye that is otherwise physically normal, or out of proportion to associated structural abnormalities...
. Very rarely, extremely large hemangiomas can cause high-output heart failure due to the amount of blood that must be pumped to excess blood vessels. Lesions adjacent to bone can also cause erosion of the bone.
The most frequent complaints about hemangiomas, however, stem from psychosocial complications: the condition can affect a person's appearance and can provoke attention and malicious reactions from others. Particular problems occur if the lip or nose is involved, as distortion can be difficult to treat surgically.
Children with large Segmental Hemangiomas of the head and neck can be associated with a disorder called PHACES Syndrome.
Treatment
Most hemangiomas disappear without treatment, leaving minimal or no visible marks, but this takes up to 3 years. Large hemangiomas can leave visible skin changes secondary to severe stretching of the skin or damage to surface texture. When hemangiomas interfere with vision, breathing, or threaten significant cosmetic injury, they are usually treated.
The mainstay of treatment is Nd:YAG laser treatment by a physician experienced in the use of these lasers for skin lesions. It is best to have the lesion treated very early when it is only a small superficial spot. Although large and deep lesions can be treated with good results, sometimes surgical excision is required. Oral
corticosteroidCorticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
therapy is an older method of treatment and usually has very limited results. Other drugs such as
interferonInterferons are natural cell-signaling proteins produced by the cells of the immune system of most vertebrates in response to challenges such as viruses, parasites and tumor cells. They belong to the large class of glycoproteins known as cytokines and are produced by a wide variety of cells in...
or
vincristineVincristine , also known as leurocristine, is a vinca alkaloid from the Catharanthus roseus , formerly Vinca rosea and hence its name. It is a mitotic inhibitor, and is used in cancer chemotherapy....
are sometimes considered if the corticosteroids do not work. If this fails, surgical removal often becomes necessary. Blockage of the airway will often require a
tracheostomyTracheotomy and tracheostomy are surgical procedures on the neck to open a direct airway through an incision in the trachea . They are performed by paramedics, veterinarians, emergency physicians and surgeons...
to be performed (insertion of an external airway through the front of the neck into the trachea below the level of the obstruction). Smaller raised lesions are sometimes treated with injection of
corticosteroidCorticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
directly into the lesion.
Beta-blocker using agents such as propranolol is a new emerging form of therapy producing some impressive responses. Recent evidence (June 2008) suggests that
propranololPropranolol is a non-selective beta blocker mainly used in the treatment of hypertension. It was the first successful beta blocker developed. It is the only drug proven effective for the prophylaxis of migraines in children...
can be used to treat severe hemangiomas. This treatment may prove superior to corticosteroids, as propranolol has far less side effects. If the initial impressions are confirmed it may become the mainstream therapy of the future.
Ulceration will usually heal with topical medication and special dressings under medical supervision. Sometimes pulsed dye laser can be used to accelerate healing.
There is also a rare effect of Hemangioma which can cause more serious complications.
Prognosis
Hemangiomas go through three stages of development and decay:
- In the proliferation stage, a hemangioma grows very quickly. This stage can last up to twelve months.
- In the rest stage, there is very little change in a hemangioma's appearance. This usually lasts until the infant is one to two years old.
- In the involution phase, a hemangioma finally begins to diminish in size. Fifty percent of lesions will have disappeared by age five with the vast majority gone by puberty.
External links