The adrenal gland is a small gland, “seated” on the kidneys. The outer part — the cortex — and is the place where they develop most cancers. The main function of the adrenal cortex — synthesis of hormones: cortisol (control metabolism), aldosterone (responsible for salt concentration in the blood and managing blood pressure) and adrenal androgens (are precursors to testosterone and estrogen). Inner brain (medullary) part of the adrenal glands is a continuation of the nervous system of the body. In it are synthesized hormones such as epinephrine and norepinephrine. Tumors of the medulla are called pheochromocytoma and neuroblastoma. This article will apply only to tumors of the adrenal cortex.
The content of the article
The location of adrenal glands there are two types of tumors of the adrenal: benign and malignant (i.e., in fact, cancer). Fortunately, in most cases the tumors are benign in nature. These tumors are called adenomas. The boundary between adenoma and malignant tumor sufficiently transparent and to understand “who is who” can only be experienced a pathologist, examining the tissue sample under a microscope. But in most cases that the tumor is malignant, it is possible to learn only after the fact of its dissemination in lymph nodes and other organs. Adenoma outside of adrenal gland are not covered.
Causes and risk factors cancer of the adrenal cortex
May bear the subtitle, the word “cause” is somewhat presumptuous, because the exact causes of cancer today, nobody knows, although research in this direction is very active. It is known that some genes (parts of our DNA) contain instructions for control of cell division and growth. Part of genes that promote cell division are called oncogenes. Other genes that slow down the division of cancer cells or causing their death, are called genes-tumor suppressors or oncosuppressor. It is also known that cancer can be caused by DNA mutations, resulting in “switching on” oncogenes or “turn off” oncosuppressor. Some mutations are hereditary, but most of them acquired in the course of the life cycle, including, as mentioned above, for some science reason.
From hereditary genetic syndromes that may affect the risk of developing cancer of the adrenal cortex include:
- Li-Fraumeni syndrome;
- the BWS Widemann;
- multiple endocrine neoplasia;
- familial adenomatous polyposis.
Non-hereditary risk factors for cancer of the adrenal cortex include:
- high diet
- a sedentary lifestyle,
- the effects of environmental carcinogens.
The symptoms and signs of cancer of the adrenal cortex
Approximately half of the patients with cancer of the adrenal glands, the symptoms associated with hormones produced by the tumor. The other half of patients suffering from symptoms caused by compression of enlarged tumor on nearby organs.
In children the symptoms of cancer of the adrenal glands caused, as a rule, androgens produced by the tumor. The most characteristic indication of this is excessive hair growth on the face and body, penis enlargement (in boys) and clitoris (girls). If the tumor produces estrogen, in girls it can cause early onset of puberty, manifested by growth of mammary glands and early menstruation (boys also may increase mammary glands). In adults the symptoms of adrenocortical carcinoma associated with high levels of sex hormones, not so noticeable.
Such symptoms are easier to detect if there is overproduction of the hormone, the opposite sex of patient: estrogens in men and androgens in women. For example, in men this may manifest itself in loss of libido and impotency in women — excessive hair growth, irregular menses, deepening of the voice.
Elevated levels of cortisol, which is also found in cancer of the adrenal glands is called Cushing’s syndrome that includes the following symptoms:
- excess weight, especially in the abdomen, collarbone, neck, shoulders (“fat pads”);
- the purple bands on the abdomen (“stretch”);
- excessive hair growth on the face, back and chest in women;
- weakness and loss of muscle mass of lower extremities;
- irregular menstruation;
- the tendency to form hematomas;
- weak bone density, osteoporosis, fractures;
- high blood pressure.
Hypersecretion of aldosterone can cause hypertension, weakness, muscle cramps and decreased potassium levels in the blood. The excess aldosterone is more often associated with adenomas of the adrenal gland, rather than with malignant tumors.
The growth of a cancerous tumor, it begins to compress adjacent organs and tissues. This can cause pain in the area of the tumor, the feeling of fullness of the stomach and the resulting problems with absorption of food.
The survival rate for cancer of the adrenal glands
Before presenting the statistics of survival for cancer of the adrenal glands National cancer Institute, need a little explanation. In their evaluation framework, the experts of the Institute rank cancer into three stages:
- localized cancer
Localized cancer is not difficult to guess, have not gone beyond their original location. Regional the cancer has spread to nearby tissue and lymph nodes, and remote — remote from the tumor tissues and organs. So, the increased five-year survival rate of patients with localized cancer is 65%, with regional — 44%, and with remote at only 7%.
Can reveal cancer of the adrenal cortex at an early stage?
Malignant tumors of the adrenal cortex is difficult to detect and diagnosed, unfortunately, being already a substantial size. In children they are easier to detect for the reason that cancer of the adrenal cortex is associated with impaired hormonal balance, it is clearly seen it is on has not yet formed body. For example, the same signs of premature maturation, which are associated with the overproduction of sex hormones. Adults have the chance to identify have breast cancer just randomly when computer tomography in conjunction with some other health problems.
Cancer of the adrenal cortex: statistics
Malignant neoplasms of the adrenal cortex are very rare. For example, in the United States every year revealed only about 300 cases. Much more frequently found benign tumors of the adrenal cortex — adenoma, typical for people of middle and old age. So, when carrying out instrumental studies (CT or MRI) adrenal adenoma is found in every tenth patient.
The average age of a patient with cancer of the adrenal cortex is 45-50 years, but this disease can come at any age, even children. Among patients with cancer of the adrenal cortex there was a slight predominance of females.
Treatment of cancer of the adrenal cortex
Let’s talk about the methods of treatment of cancer of the adrenal cortex, including surgery, radiation therapy, chemotherapy and support drug therapy.
Surgery to remove the adrenal gland is called adrenalectomy. There are two basic approaches to conducting this surgery. In the first case, the surgeon makes an incision in the back directly underneath the ribs. This technique is used only for tumors of small size. For larger tumors, the incision is made in the abdominal wall that allows to see the location of the tumor and whether it has spread. Another advantage of this technique — you can remove nearby organs and tissues affected by the tumor. For example, if the cancer is “sprouted” in the kidney or the liver, the affected part of the body must be completely removed. If the tumor has penetrated into the muscles and fatty tissue — these tissues must also be subjected to resection.
Sometimes the tumor penetrates into the inferior Vena cava is a large vessel that carries blood from the lower body to the heart. In this case, you want a large-scale operation whose purpose is the removal of the tumor and protection of Vienna from damage. Throughout the surgery the patient is connected to system of artificial circulation.
Tumors of small size can be removed and the laparoscopic method. A laparoscope is a hollow thin tube with a microscopic camera on the end which is introduced into the body through a small incision. Other tools required for the immediate removal of the tumor, are inserted through the laparoscope or through other small incisions. The main advantage of this method is its atraumatically that shortens recovery time after surgery.
Despite the fact that laparoscopy is mainly used for removal of adenomas, can be applied at a sufficiently large malignant tumors. However, given that the tumor should be completely removed, it is pre-cut into several small parts. The risk of the cancer spreading increases, so this method is not used in the advanced stages of cancer when the tumor has penetrated nearby organs and lymph nodes.
Radiation therapy involves the use of high doses of radiation to kill cancer cells. In distance method, the radiation is supplied by an external source. Sessions are held daily 5 days a week for several weeks. Each session lasts only a few minutes and like normal x-rays. Before you reach tumors, radiation passes through the skin and other tissues, but the actual exposure time is very small, and its focus is very accurate, so the negative effects are not as impressive, although, of course, are the place to be.
Brachytherapy is a form of internal radiation where the radioactive material in the form of small grains is placed near the tumor or directly into it, and sometimes thin plastic tubes. Tubes of radioactive material are left for a few days inside and then removed. The amount of time before removal of the tubes is determined by the capacity of radioactive substances and size of the tumor.
Radiation therapy is not often used as the primary treatment for cancer of the adrenal cortex, because cancer cells are quite resistant to x-ray irradiation. This method can be used after surgery to prevent cancer recurrence. Radiation therapy is also used for influencing the field of cancer metastasis, such as bone and brain.
Chemotherapy should be used on advanced stages of the disease, when the cancer has spread to distant from the tumor tissues and organs. This method is not the best choice for cancer of the adrenal cortex: it is used only when the tumor cannot be removed surgically. By and large, chemotherapy does not cure cancer.
As chemotherapeutic agents in cancer of the adrenal cortex most often used Mitotan. This drug blocks the secretion of adrenal hormones and destroys the tumor cells and healthy tissue of the adrenal glands. This leads to lower levels of cortisol and other hormones, which causes weakness and General malaise. This effect is offset by the administration of steroid hormones, restoring the faltering hormonal balance. Mitotane can affect the blood concentration of other hormones such as thyroid hormones and testosterone. In these cases also shows hormone replacement therapy.
Sometimes Mitotane administered after surgical removal of the entire visible part of the tumor, if there is a chance that the body remained cancer cells. If the tumor was not completely removed or had a relapse, Mitotan can produce the desired effects in 30% of cases. Its deterrent effect on cancer lasts on average about a year.
Mitotan especially good for those patients who suffer from hormonal hypersecretion. Even if he does not reduce the size of the tumor, Mitotane will reduce the level of adrenal hormones and lessen the associated symptoms. Efficiency Mitotane in the circumstances is 80%.
Another option for chemotherapy for cancer of the adrenal cortex to receive the drug combination of Cisplatin, Doxorubicin and Etoposide. Among other options can be considered Paclitaxel, 5-Fluorouracil, Vincristine and Streptozocin.
Along with Mitotane, to reduce the level of hormones of the adrenal cortex may be used and other medications not related to chemotherapy. These questions are not oncologists, and endocrinologists, who should also carefully examine the patient’s response to certain hormones, which in themselves can contribute to imbalances in the hormonal system, and without cancer.
Ketoconazole, Aminoglutethimide and Metyrapone — that is drugs that reduce the secretion of hormones of the adrenal cortex. They can ease symptoms caused by hormonal imbalance, but does not affect the size of the tumor.
A number of drugs blocks the effects of hormones produced by the tumor itself:
- Spironolactone (Aldactone), which reduces the level of aldosterone;
- Mifepristone (Korlem), decreasing the concentration of cortisol;
- Tamoxifen, Fareston and Faslodex, which is a estrogen blocker. These drugs are usually used in breast cancer but may be useful for patients suffering from estrogenprogestogen tumors of the adrenal cortex.
Emotional state after treatment
When treatment is over, you can experience the whole kaleidoscope of various emotions. It happens to every person struggling with cancer, there’s nothing unusual. You often start to think about death. Or, you may begin to protect your family members, worry about friends, co-workers, who also may face this serious disease. It is possible that you will revise the whole system of values and relationships with others. It may be that anxiety will be overcome in the most unexpected situations. For example, as you feel better, you’ll be less likely to see your doctor. Some cancer patients, this fact can cause almost panic.
The most important thing is to understand that life continues and to enjoy each day of my life: in this case we can say that you won.