Hemangioma

hemangiomaHemangioma is a benign vascular neoplasm of the skin widely known as a birthmark or internal organs.

Description
Hemangioma consists of the bunch of abnormal  vessels and usually appear on the skin, but some may be found in the internal organs as well. These tumors are the most common neoplasms affecting the children. Infantile hemangiomas occur in 10% of the population and they are present at birth (about in 33% of cases) or appear in a few weeks after the birth. Commonly hemangiomas disappear by the age of 9 spontaneously, although some of them may leave residual teleangiectasia, scars or pigmented/depigmented areas.

Causes
The cause of the hemangioma formation is not known, although some in some families infants tend to have birthmarks. Autosomal dominant inheritance is suggested. There are several theories of what may cause the blood vessels proliferation. According to one theory, the hemangioma endothelial cells originate from disrupted placental tissue that penetrates the soft tissues of the fetus

during gestation or birth. The second theory suggests that the cells of hemangioma arise from the stem cells of the affected person.

Probably estrogen signaling is vital for the vessel proliferation and causes formation of the vessel tumors.


Risk factors
Females are more likely to have hemangiomas; prematurity, twin birth and low birth weight are also considered to be risk factors for hemangiomas. The frequency of hemangioma is higher among the Caucasians.  

Symptoms
Cutaneous hemangiomas usually occur on the head and neck, less commonly they can be found on the trunk or extremities. Extracutaneous hemangiomas may affect almost every internal organ – usually it’s the liver, gastrointestinal tract and central nervous system, pancreas, larynx, and etc. Capillary hemangiomas usually appear in the top layers of the skin, while cavernous hemangiomas arise in the deeper layers.

Hemangioma on the skin appear as well-demarcated flat red erythematous to reddish-purple patch. Hemangioma can also be raised over the skin surface like papules. It proliferates rapidly and grow soon, this stage lasts about three months. Sometimes the development of hemangioma causes ischemia, necrosis, ulceration, and bleeding in the affected area. Ulceration usually occur the posterior scalp, neck folds and perianal areas.These hemangiomas look like black crusted plaques or papules or painful erosions. Later bleeding may occur. The next stage is called quiescence. During this phase hemangioma stop progressing or grows slowly. The overlying skin becomes grayish.

Deep hemangiomas appear as bluish macules that can turn into nodules or large tumors.

Capillary hemangiomas of the eye may cause decreased vision (amblyopia).

Depending on the extent of the neoplasms hemangiomas are classified as focal/localized, segmental and indeterminate. Focal hemangiomas are typically sole and appear in the concrete area. Segmental hemangioma cover the larger area of the skin and demand further examination, because they may be the symptom of another disorder.

[Read also: Maffucci syndrome]

Diagnosis
The physical examination helps evaluate the diagnosis. If neoplasm is located in the body or internal organs ultrasound with Doppler, CT or MRI is necessary. Biopsy of the lesion may be performed.

If the person has more than 5 hemangiomas it is strongly recommended to examine him/her precisely, because internal organs may also be affected.

Treatment
Small neoplasms doesn’t require any treatment as long as almost half of the hemangiomas disappear by the age of 5, by the age of 9 all of them usually vanish.
The size of the neoplasm may be reduced by the administration of beta-blocker medicines (propranolol).

Laser treatment may be needed if the tumor interferes with the vision (if the eye is affected). Large cavernous hemangioma may be treated by the intake of steroids by mouth or injections into the neoplasm. Steroids cause the shrinkage of the abnormal vessels.

Some hemangiomas require surgical removal.

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