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Adrenal Cortical Carcinoma

Adrenal Cortical Carcinoma – General Information

Adrenal Cortical Carcinoma, also known as Adrenocortical Carcinoma or Adrenal Cortex Cancer, is considered to be a painful cancer, which has its origins in the steroid hormone producing tissue of the adrenal gland called cortex. This type of cancer is a rarely and has a bimodal distribution by age, and can be encountered in children under six years, but also in adults between 30 and 40 years old. The Adrenal Cortical Carcinoma is a disease caused by the hormones and can occur in persons with steroid and hormone producing tumors, and can be easily confused with  Cushing's syndrome, Conn syndrome, virilization, and feminization. The cancer is considered an aggressive one, because can invade the nearby tissues or can metastasize to other organs at the time of the diagnosis, which leads to a low survival rate.

Adrenal Cortical Carcinoma has commonly four stages, and each stage has its characteristics. In the first stage, the adrenal gland presents no local invasion or any distant metastases and the tumor diameter has less than 5 cm. The second stage of the cancer presents almost all the characteristics like the first one, but this time, the tumors diameter is greater than 5 cm. In the third stage of the cancer, the local invasion takes place, but doesn’t affect the nearby organs or the regional lymph nodes. The distant metastases occur in the forth stage of the adrenal cancer, and the invasion takes place into the adjacent organs and the nearby lymph nodes.

Adrenal Cortical Carcinoma – Symptoms

The symptoms of Adrenal Cortical Carcinoma are different in children and adults. The tumors found in children are in most of the cases functional and the virilization is the most common symptom of the cancer, which follows the precocious puberty. Most of the patients that are diagnosed with Adrenal Cortical Carcinoma are carefully examined for any signs and symptoms of this hormonal cancer. The symptoms of this cancer are, frequently, the same as the other hormonal syndromes, like Cushing's syndrome and include weight gain, purple stripes on the abdomen, muscle wasting, a swallow on the neck called the “buffalo hump”, a moonlike face, a thinning skin which becomes very fragile. Another hormonal syndrome that has the same symptoms like the cancer is virilism, which is caused by androgen excess, and is most encountered in women, and includes excess facial and body hair, acne, deepening of the voice, enlargement of the clitoris, coarsening of facial features, and also cessation of menstruation. The Conn syndrome, which is caused by a mineralocorticoid excess, has also some familiar symptoms with the adrenal cancer, such as high blood pressure, which can lead to headache, and hypokalemia, a low serum potassium level, which can lead to muscle weakness, confusion, and palpitations, low plasma renin activity, and high serum aldosterone. But, there are some rare cases, in which the feminization syndrome takes place, caused by a estrogen excess, and it is encountered especially in men, and includes breast enlargement, decreased libido and also impotence.

Adrenal Cortical Carcinoma – Treatment

This type of cancer is treated by a stage treatment, which consists in a trial of 4 stages. Usually, for the first and second stages of the treatment in Adrenal Cortical Carcinoma, the main treatment applied is surgery, when the adrenal gland is removed. There are two adrenal glands and the removal of the abnormal one does not cause any problems for the patient. The lymph nodes that surround the glands are in this case enlarged and are removed at the same time of surgery for examination. Most doctors don’t remove these lymph nodes, but only if the size of the nodes are normal. The treatment is no more necessary if the cancer does not producing any hormones at all. But, there will be periodic examinations since even stage I cancers can come back.

The third stage of the treatment also includes surgery to treat Adrenal Cortical Carcinoma, also if the cancer invaded the area around and the lymph nodes. In this stage, the doctor will attempt to remove the abnormal adrenal gland and as much as possible of the cancer, but he can also remove any affected organs nearby, such as kidney or a part of the liver. Like in the first and second stages, the lymph nodes that surround the glands are also removed in the surgery process, for examination also.

In the forth stage, even when Adrenal Cortical Carcinoma has spread to other parts of the body and in most of the cases can not be cured, and by surgery the only option is to remove as much of the tumor that can be removed. This procedure is usually called debulking. By removing a part of the cancer this will reduce the production of hormones is less which can be causing symptoms. Radiation therapy can also be used to treat affected areas of remaining cancer that are also causing any symptoms, like people that suffer with painful bone metastases. Mitotane therapy can be applied before the surgery, but the doctors can also wait until the appearance of symptoms and can be used in combination with chemotherapy.

For the recurrent for of Adrenal Cortical Carcinoma, the treatment is almost the same like in the forth stage and to get off the symptoms, surgery is applied, but the most of the patients are treated with mitotane. The patient can also chose the chemotherapy and radiation therapy for the treatment. Drugs are another option to take in consideration, but only if the mitotane treatment isn’t tolerated and the patient still has symptoms produced by the hormones from the tumor, to lower the hormone production. In most of the cases, these types of treatment provide a temporary improvement, because the tumor keeps growing. When this happens palliative treatment is applied, and it is considered a better choice to improve the health, because the other treatments are no longer effective. To relieve the pain the doctors also use morphine and some narcotic drugs, but there is a strict restriction in prescribing morphine, because there can appear the addiction.




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