Hemangioma is a developmental malformation of blood vessels and not a typical tumor. Hemangioma is often present since birth and it never turns malignant.
Types of Hemangiomas
There are mainly three types of hemangioma
- Capillary hemangioma – arising from capillaries.
- Venous or cavernous hemangioma – arising from veins.
- Arterial or plexiform hemangioma – arising from arteries.
A hemangioma may occur anywhere in the body though it is more common in the skin and subcutaneous tissues.
Capillary Hemangioma
Four varieties of capillary hemangioma are seen
- Salmon patch
- Port-Wine stain
- Strawberry angioma
- Vin Rose patch.
Spider naevus, a type earlier included in capillary hemangioma actually arises from skin arteriole and is not a true capillary hemangioma.
Salmon Patch
This is present since birth.
It usually disappears before the first birthday. It is mostly seen over the middle of the forehead, or occiput or anywhere in the midline.
Port-Wine Stain
It is usually present since birth and does not show any change for the rest of life. The size gradually varies in proportion to the whole body.
Common on the face, at the shoulders, neck, and buttock.
It is deep purple-red in color which may become paler in later life.
It is a diffuse vascular deformity. It may become nodular in some areas.
With pressure, the color diminishes in intensity, but when the pressure is released, the color of the naevus takes time to reappear as the feeding vessels are also abnormal.
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Strawberry Angioma
Red mark is noticed after 1 to 3 weeks of birth that gradually increases in size; comparatively rapidly for a few weeks or months till it becomes a typical strawberry or raspberry-like swelling.
After the first birthday, the angioma gradually regresses in size and may disappear by the age of 7 to 8 years.
The subcutaneous tissue, as well as the skin, is often involved. Sometimes muscles may be affected. Very rarely submucous strawberry angioma has been seen when it is prone to alarming hemorrhage.
The lesion is composed of immature vascular tissue.
This angioma is usually 1 to 2 cm in diameter, but this may become as large as up to 8 cm in diameter. Strawberry angioma is soft, compressible and not pulsatile. Sustained pressure will squeeze most of the blood out of the haemangioma leaving it collapsed. As soon as the pressure is released, refilling occurs quickly. It is usually seen in the skin and is freely mobile over the skin.
Vin Rose Patch
It is a congenital intradermal vascular abnormality in which there is mild dilatation of vessels in the skin. This lesion takes a pale pink color.
It may be associated with other vascular abnormalities e.g. extensive haemangiomata, arterio-venous fistula in the limb leading to giant limb lymphoedema, etc.
This lesion can occur anywhere in the body. It does not cause any symptom. It is not dark enough to produce disfigurement. It is often accepted as a minor birthmark and is of the forgotten about.
Treatment of Capillary Hemangioma
Majority of the capillary haemangiomas disappear on their own. So it is worthwhile to wait and watch.
If the lesion exists even after 8 years and the patient and the parents insist on treatment for cosmetic reason, the following are the treatment options available
- Excision of the lesion with skin grafting.
- Carbon dioxide snow application.
- Injection of hot water or hypertonic saline or sclerosing agent
- X-ray therapy has not proved very successful, yet may be considered in failure cases though it may cause disturbances of growth, necrosis of skin, pigmentation, and ulceration.
- Injection of steroid.
CO2 or ND Yag Laser is now the treatment of choice when this expertise is available. This treatment is particularly useful in port-wine stain.
Osler-Weber-Rendu Disease
This is also called hereditary hemorrhagic telangiectasia, which is characterized by multiple small aneurysmal telangiectases distributed over the skin and mucous membrane of the body. These are often present from birth and are hereditary in origin which is transmitted by dominant Mendelian trait.
Twenty percent of cases lack family history.
Lesions are found beneath the skin in any region of the body or directly beneath the mucous membrane of the oral cavity, alimentary tract, respiratory tract, and urinary tract and even in the brain, liver, and spleen.
Individual lesions are smaller than 5 mm in diameter, but often coalesce to produce lesions up to several centimeters in diameter which are reddish-blue in color.
This case presents with hemorrhage from rupture of these lesions either into the intestinal tract, urinary or respiratory tract, nose bleeding etc.
It is a relatively rare condition.
Sturge-Weber syndrome
It is characterized by facial port-wine stain angioma along with ipsilateral venous angiomas in the leptomeninges over the cerebral hemisphere.
It is often associated with mental retardation and attacks of Jacksonian epilepsy.
Cavernous Hemangioma
It consists of multiple dilated venous channels. It is a spongy swelling and is usually present since birth.
It does not show any tendency to involution. On the contrary, it may become larger and more troublesome as time goes on.
These are always raised from the surface and are localized swellings, spongy in consistency.
These are bluish in color as the content is venous blood.
These are non-pulsatile. If pulsatile, communication with arterial system (arteriovenous fistula) should be suspected.
Compressibility can be seen. Continued pressure and squeezing will drive the blood out of the lesion and the swelling crumbles. As soon as the pressure is removed, swelling reappears with refilling.
Common sites are
- face, cheek, ears
- mucous membrane of lips, mouth, and tongue,
- organs like liver, kidney an, brain.
Complications
Cavernous angioma may be associated with a lipoma (naevolipoma).
In some cases, arteriovenous communications (arteriovenous fistula) may be present.
The skin overlying the angioma may be atrophic and may cause severe hemorrhage from trauma.
If organisms gain entry into this angioma, they grow rapidly and may produce septicemia.
Sometimes calcification may occur in the form of calcified nodules (phleboliths) in this angioma.
Very rarely cavernous haemangioma may turn malignant to produce haemangiosarcoma.
Treatment
Conservative treatment is more often required in the form of:
- Injection of a sclerosing agent into the lesion. In this respect, 3% sodium morrhuate is quite effective otherwise boiling water or hypertonic saline may be tried. The injection is given once a week for a few times up to 6 weeks, if necessary.
- Cautery treatment may be applied to the haemangioma. A needle is pushed into the haemangioma and its end is touched with a diathermy node.
Surgery is a better treatment if the swelling is small and localized. The feeding vessels are first ligated and the whole lesion is excised. Diathermy may be used to control hemorrhage. Such excision may be made easier with preliminary conservative treatment.
If there is a feeding artery, which is revealed by arteriography, therapeutic embolization is quite successful. Application of laser has been claimed to be successful in this condition.
Arterial or Plexiform Hemangioma
It is a type of congenital arteriovenous fistula. There is a pulsatile swelling of arteries and the veins become arterialized i.e. tortuous and thick walled and pulsatile-feeling like a bag of pulsating earthworms. This is often called cirsoid aneurysm. Such cirsoid aneurysm is commonly seen on the forehead or on the scalp over the temporal region.
Characteristic features
- A diffuse swelling is more often seen in the forehead or scalp over the temporal region.
- The swelling feels like a bag of pulsating earthworms.
- The swelling is pulsatile. It is also compressible.
- On auscultation one may hear bruit or systolic thrill over the swelling.
- On skiagraphy one may find pressure effect of this lesion on the underlying bone in the form of indentation and osteoporosis.
Treatment
- Ligation of feeding vessels.
- Therapeutic embolization of the feeding artery.
- After ligation of feeding vessels, excision of the lesion with diathermy.
Spider naevus
It is a solitary dilated skin arteriole feeding a number of small branches which grow in a radial manner.
It is an acquired condition and is associated with some generalized disease. Such spider naevus appears on the upper half of the trunk, face, and arms. Peculiarly enough this is the drainage area of superior vena cava through its relationship cannot be explained.
Spider naevus fades completely when compressed with the finger.
Spider naevus is usually associated with hepatic disease (particularly hepatic cirrhosis or tumors destroying the liver) and tumors producing estrogen.
Cavernous Haemangioma In Vascular Nevi
It is constituted by much larger vascular spaces which are lined by a single layer of endothelial cells. The lesions are usually present at birth and do not show any significant increase in size later. Clinically, they consist of red or bluish red irregular, lobulated masses which may be situated on any part of the body.
Surface lesions may manifest as reddish or bluish vesicles when situated near the eye or on the tongue, they may cause pressure symptoms and interfere with the function of the part. When situated on the pressure points such as the knee or the elbow, they may get easily traumatized and result in profuse bleeding.
Treatment
The treatment of these lesions also lies in surgical excision. The use of diathermic knife or electro-surgical excision is preferable because during surgical excision such lesions can bleed profusely.
Other methods of treatment include deep X-ray therapy or intralesional injection of sclerosing agents such as boiling water or five percent phenol in almond oil. Such treatments produce inflammation in the lesion which results in fibrosis, scarring, and occlusion of the blood spaces. Cryotherapy or lasers can also be used to destroy such lesions.