Gigantism is an extremely rare disease that contributes to the growth disorders in children. It appears when the pituitary gland produces too large amounts of growth hormone, known as somatotrophin.
The causes of gigantism
In most cases, the cause of gigantism are irregularities in the functioning of the pituitary tumor. This gland is the size of a pea and is located at the base of the brain. It influences the regulation of many functions in the body as temperature control, sexual development, growth, metabolism and urine production. When the tumor compresses the pituitary gland, it begins to produce much more growth hormone than the body needs it.
Among the less common causes of gigantism, may be mentioned:
- McCune-Albright syndrome, which affects the abnormal growth of bone tissue, changes the tint of the skin to light brown and causes the gland disorders;
- Carney complex – a hereditary disease that causes non-cancerous nodules;
- MEN1 syndrome – an inherited disorder causing tumors in the pituitary gland, pancreas, and parathyroid glands;
- neurofibromatosis – a hereditary disease, which contributes to the formation of tumors in the nervous system.
The symptoms of gigantism
Among the most characteristic symptom of the disease is significantly larger child’s growth – particularly compared to its peers, and excessive growth of certain body parts, which are evidently more massive in comparison to the others. Typical symptoms include very large hands and feet, thick fingers, prominent jaw and forehead, coarse facial features.
Children suffering from gigantism also have flat noses, large heads, lips, and tongue.
Symptoms may also include headaches vision abnormalities, nausea, or cancer, especially when the growing pituitary tumor pressing on the nerves of the brain.
May also happen excessive sweating, severe and recurrent headaches weakness sleeping problems, delayed puberty, irregular menstrual periods among girls and the deafness.
Damage to the pituitary gland can lead to lower levels of other hormones like cortisol, estradiol in girls, testosterone in boys or thyroid hormones.
Treatment of gigantism
After diagnosing the disease, the most common treatment is based on the efforts to stop or slow production of growth hormone.
The main method of treatment of gigantism is surgical removal of the tumor, which is held through the incision to the nose. By using microscopic cameras, the procedure is minimally invasive, and usually, on the second day after the surgery, a child can leave the hospital safely.
Pharmacotherapy is used when surgery is not possible. In cases, where there is a high risk of serious damages to the blood vessels or nerves. Most often recommended medications are octreotide or lanreotide long acting that prevents the release of growth hormone. Most often it is used as an injection, once a month.
Other well-known medications are oral drugs in tablet form as bromocriptine or cabergoline, which help to reduce the level of growth hormone. In situations where the drugs do not produce measurable results, in addition, is used the pegvisomant, which blocks the action of somatotropin.
The last of the known methods is the radio-surgical knife Gamma – highly concentrated rays beams that can effectively destroy the tumor without damaging the surrounding healthy tissue. Very often the treatment with this method takes years (5-10 years), it requires a full effectiveness in a stabilization of growth hormone in the body, and at the same time, almost always, it affects other body hormones. Among the side effects of radiation therapy, can be problems with obesity, learning disabilities or emotional problems, therefore, it belongs to the methods of treatment used only eventually.