Hemangioma
Encyclopedia
A hemangioma of infancy is a benign
self-involuting
tumor
(swelling or growth) of endothelial
cells, the cells that line blood vessel
s. It usually appears during the first weeks of life and sometimes resolves by age 10. In more severe case hemangioma may have permanency, if not treated by a physician. Laser surgery is the most proactive way of removal in cases where patients are older than 10 years of age. In infancy, it is the most common tumor. The word "hemangioma" comes from the Greek haema- (αίμα), "blood"; angeio (αγγείο), "vessel"; -oma (-ωμα), "tumor".
-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
tumors, growing lesion
s 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 stain
s (capillary
vascular malformation) and masses of abnormal swollen vein
s (venous malformations).
and filled with blood
. The appearance depends on location. If they are on the surface of the skin, they are reminiscent of a ripe strawberry (hence, they are sometimes referred to as "strawberry hemangiomas"); however, 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. Some are formed during gestation
; the most common are not congenital, but appear during the first few weeks of life. They are often initially misdiagnosed as a scratch or bruise
; but the correct 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 Caucasian
s, and are less prevalent in other ethnicities. Females are three to five times as likely to have hemangiomas as males. Hemangiomas 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.
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,. However, researchers at Duke University conducted genetic analyses of single-nucleotide polymorphism in hemangioma tissue compared to the mother's DNA
that contradicted this hypothesis. 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.
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 eye
s, causing an occlusion amblyopia
. 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. The potential for psychological injury develops from school age onward. It is therefore important to consider treatment prior to school if adequate spontaneous improvement has not occurred.
Children with large segmental hemangiomas of the head and neck can be associated with a disorder called PHACES Syndrome.
Until recently, the mainstay of treatment was oral corticosteroid
therapy, but there are now alternative treatments. A randomized trial showed that the beta-blocker propranolol reduced severe hemangiomas in infants. The topically applied beta blocker
solution / gel Timolol
, is also being trialled for small facial hemangiomas that do not justify systemic treatment. Other treatments that have been used include interferon
or vincristine
. They may be considered if first-line therapy fails.
Surgical removal is sometimes indicated, particularly if there has been delay in commencing treatment and structural changes have become irreversible. Surgery may also be necessary to correct distortion of facial features, again in the case of inadequate or failed early medical intervention. Blockage of the airway will often require a tracheostomy
to be performed, which involves the 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 corticosteroid directly into the lesion. A pulsed dye laser
can be useful for very early, flat, superficial lesions, if they appear in cosmetically significant areas or for those lesions that leave residual surface blood vessels in the case of incomplete resolution. Sometimes a pulsed dye laser can be used to accelerate healing. Unfortunately, raised lesions or lesions under the skin do not respond to laser treatment. Ulceration will usually heal with topical medication and special dressings under medical supervision.
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
self-involuting
Involution (medicine)
-Thymus:The thymus continues to grow between birth and puberty and then begins to atrophy, a process directed by the high levels of circulating sex hormones. Proportional to thymic size, thymic activity is most active before puberty. Upon atrophy, the size and activity are dramatically reduced,...
tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
(swelling or growth) of endothelial
Endothelium
The endothelium is the thin layer of cells that lines the interior surface of blood vessels, forming an interface between circulating blood in the lumen and the rest of the vessel wall. These cells are called endothelial cells. Endothelial cells line the entire circulatory system, from the heart...
cells, the cells that line blood vessel
Blood vessel
The blood vessels are the part of the circulatory system that transports blood throughout the body. There are three major types of blood vessels: the arteries, which carry the blood away from the heart; the capillaries, which enable the actual exchange of water and chemicals between the blood and...
s. It usually appears during the first weeks of life and sometimes resolves by age 10. In more severe case hemangioma may have permanency, if not treated by a physician. Laser surgery is the most proactive way of removal in cases where patients are older than 10 years of age. In infancy, it is the most common tumor. The word "hemangioma" comes from the Greek haema- (αίμα), "blood"; angeio (αγγείο), "vessel"; -oma (-ωμα), "tumor".
Terminology
The terminology used to define, describe and categorize vascular anomalies, abnormal lumps made up of blood vessels, has changed. The term hemangioma was originally used to describe any vascular tumorTumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
-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
Involution (medicine)
-Thymus:The thymus continues to grow between birth and puberty and then begins to atrophy, a process directed by the high levels of circulating sex hormones. Proportional to thymic size, thymic activity is most active before puberty. Upon atrophy, the size and activity are dramatically reduced,...
tumors, growing lesion
Lesion
A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...
s 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 stain
Port-wine stain
A port-wine stain or naevus flammeus is a vascular anomaly consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discoloration of the skin. They are so called for their colour, resembling that of port wine...
s (capillary
Capillary
Capillaries are the smallest of a body's blood vessels and are parts of the microcirculation. They are only 1 cell thick. These microvessels, measuring 5-10 μm in diameter, connect arterioles and venules, and enable the exchange of water, oxygen, carbon dioxide, and many other nutrient and waste...
vascular malformation) and masses of abnormal swollen vein
Vein
In the circulatory system, veins are blood vessels that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenated blood to the heart...
s (venous malformations).
Signs and symptoms
Hemangiomas are connected to the circulatory systemCirculatory system
The circulatory system is an organ system that passes nutrients , gases, hormones, blood cells, etc...
and filled with blood
Blood
Blood is a specialized bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells....
. The appearance depends on location. If they are on the surface of the skin, they are reminiscent of a ripe strawberry (hence, they are sometimes referred to as "strawberry hemangiomas"); however, if they are just under the skin they present as a bluish swelling. Sometimes they grow in internal organs such as the liver
Liver
The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...
or larynx
Larynx
The larynx , commonly called the voice box, is an organ in the neck of amphibians, reptiles and mammals involved in breathing, sound production, and protecting the trachea against food aspiration. It manipulates pitch and volume...
. In most cases, hemangiomas will disappear over time. Some are formed during gestation
Gestation
Gestation is the carrying of an embryo or fetus inside a female viviparous animal. Mammals during pregnancy can have one or more gestations at the same time ....
; the most common are not congenital, but appear during the first few weeks of life. They are often initially misdiagnosed as a scratch or bruise
Bruise
A bruise, also called a contusion, is a type of relatively minor hematoma of tissue in which capillaries and sometimes venules are damaged by trauma, allowing blood to seep into the surrounding interstitial tissues. Bruises can involve capillaries at the level of skin, subcutaneous tissue, muscle,...
; but the correct diagnosis
Diagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
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
Pallor
Pallor is a reduced amount of oxyhaemoglobin in skin or mucous membrane, a pale color which can be caused by illness, emotional shock or stress, stimulant use, lack of exposure to sunlight, anaemia or genetics....
. Sometimes they present as a flat red or pink area. Hemangiomas are the most common childhood tumor, occurring in approximately ten percent of Caucasian
Caucasian race
The term Caucasian race has been used to denote the general physical type of some or all of the populations of Europe, North Africa, the Horn of Africa, Western Asia , Central Asia and South Asia...
s, and are less prevalent in other ethnicities. Females are three to five times as likely to have hemangiomas as males. Hemangiomas are also more common in twin
Twin
A twin is one of two offspring produced in the same pregnancy. Twins can either be monozygotic , meaning that they develop from one zygote that splits and forms two embryos, or dizygotic because they develop from two separate eggs that are fertilized by two separate sperm.In contrast, a fetus...
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 estrogenEstrogen
Estrogens , oestrogens , or œstrogens, are a group of compounds named for their importance in the estrous cycle of humans and other animals. They are the primary female sex hormones. Natural estrogens are steroid hormones, while some synthetic ones are non-steroidal...
signaling in hemangioma proliferation. In 2007, a paper from the Stanford Children's Surgical Laboratory revealed that localized soft tissue hypoxia
Hypoxia (medical)
Hypoxia, or hypoxiation, is a pathological condition in which the body as a whole or a region of the body is deprived of adequate oxygen supply. Variations in arterial oxygen concentrations can be part of the normal physiology, for example, during strenuous physical exercise...
coupled with increased circulating estrogen after birth may be the stimulus. There is also a hypothesis
Hypothesis
A hypothesis is a proposed explanation for a phenomenon. The term derives from the Greek, ὑποτιθέναι – hypotithenai meaning "to put under" or "to suppose". For a hypothesis to be put forward as a scientific hypothesis, the scientific method requires that one can test it...
presented by researchers at Harvard and the University of Arkansas that maternal placenta
Placenta
The placenta is an organ that connects the developing fetus to the uterine wall to allow nutrient uptake, waste elimination, and gas exchange via the mother's blood supply. "True" placentas are a defining characteristic of eutherian or "placental" mammals, but are also found in some snakes and...
embolizes to the fetal
Fetus
A fetus is a developing mammal or other viviparous vertebrate after the embryonic stage and before birth.In humans, the fetal stage of prenatal development starts at the beginning of the 11th week in gestational age, which is the 9th week after fertilization.-Etymology and spelling variations:The...
dermis
Dermis
The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular dermis...
during gestation resulting in hemangiomagenesis,. However, researchers at Duke University conducted genetic analyses of single-nucleotide polymorphism in hemangioma tissue compared to the mother's DNA
DNA
Deoxyribonucleic acid is a nucleic acid that contains the genetic instructions used in the development and functioning of all known living organisms . The DNA segments that carry this genetic information are called genes, but other DNA sequences have structural purposes, or are involved in...
that contradicted this hypothesis. 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, called ulceration. If the ulceration is deep, significant bleedingBleeding
Bleeding, technically known as hemorrhaging or haemorrhaging is the loss of blood or blood escape from the circulatory system...
may occur in rare occasions. Ulceration on the diaper area can be painful and problematic. If a hemangioma develops in the larynx
Larynx
The larynx , commonly called the voice box, is an organ in the neck of amphibians, reptiles and mammals involved in breathing, sound production, and protecting the trachea against food aspiration. It manipulates pitch and volume...
, breathing
Breathing
Breathing is the process that moves air in and out of the lungs. Aerobic organisms require oxygen to release energy via respiration, in the form of the metabolism of energy-rich molecules such as glucose. Breathing is only one process that delivers oxygen to where it is needed in the body and...
can be compromised. A hemangioma can grow and block one of the eye
Human eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
s, causing an occlusion amblyopia
Amblyopia
Amblyopia, also known as lazy eye, is a disorder of the visual system that is characterized by a vision deficiency in an eye that is otherwise physically normal, or out of proportion to associated structural abnormalities of the eye...
. 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
Bone
Bones are rigid organs that constitute part of the endoskeleton of vertebrates. They support, and protect the various organs of the body, produce red and white blood cells and store minerals. Bone tissue is a type of dense connective tissue...
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. The potential for psychological injury develops from school age onward. It is therefore important to consider treatment prior to school if adequate spontaneous improvement has not occurred.
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. 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.Until recently, the mainstay of treatment was oral corticosteroid
Corticosteroid
Corticosteroids 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, but there are now alternative treatments. A randomized trial showed that the beta-blocker propranolol reduced severe hemangiomas in infants. The topically applied beta blocker
Beta blocker
Beta blockers or beta-adrenergic blocking agents, beta-adrenergic antagonists, beta-adrenoreceptor antagonists or beta antagonists, are a class of drugs used for various indications. They are particularly for the management of cardiac arrhythmias, cardioprotection after myocardial infarction ,...
solution / gel Timolol
Timolol
Timolol maleate is a non-selective beta-adrenergic receptor blocker.-Uses:In its oral form , it is used:* to treat high blood pressure* to prevent heart attacks* to prevent migraine headaches...
, is also being trialled for small facial hemangiomas that do not justify systemic treatment. Other treatments that have been used include interferon
Interferon
Interferons are proteins made and released by host cells in response to the presence of pathogens—such as viruses, bacteria, or parasites—or tumor cells. They allow communication between cells to trigger the protective defenses of the immune system that eradicate pathogens or tumors.IFNs belong to...
or vincristine
Vincristine
Vincristine , formally known as leurocristine, sometimes abbreviated "VCR", 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.-Mechanism:Tubulin is a structural protein that polymerizes to...
. They may be considered if first-line therapy fails.
Surgical removal is sometimes indicated, particularly if there has been delay in commencing treatment and structural changes have become irreversible. Surgery may also be necessary to correct distortion of facial features, again in the case of inadequate or failed early medical intervention. Blockage of the airway will often require a tracheostomy
Tracheotomy
Among the oldest described surgical procedures, tracheotomy consists of making an incision on the anterior aspect of the neck and opening a direct airway through an incision in the trachea...
to be performed, which involves the 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 corticosteroid directly into the lesion. A pulsed dye laser
Dye laser
A dye laser is a laser which uses an organic dye as the lasing medium, usually as a liquid solution. Compared to gases and most solid state lasing media, a dye can usually be used for a much wider range of wavelengths. The wide bandwidth makes them particularly suitable for tunable lasers and...
can be useful for very early, flat, superficial lesions, if they appear in cosmetically significant areas or for those lesions that leave residual surface blood vessels in the case of incomplete resolution. Sometimes a pulsed dye laser can be used to accelerate healing. Unfortunately, raised lesions or lesions under the skin do not respond to laser treatment. Ulceration will usually heal with topical medication and special dressings under medical supervision.
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. 50% of lesions will have disappeared by 5 years of age, and the vast majority will have gone by 10
External links
- Hemangioma - Children's Hospital Los Angeles
- Hemangioma - at Web MD
- Hemangioma - Children's Hospital Boston
- Humpath #1990 (Pathology images) at humpath.com
- Hepatic Hemangioma at medicinenet.com
- Haemangiomas of infancy at rch.org.au
- Liver Hemangioma at USUHS - MedPix