Today's blog post is on andrenocortical carcinoma. This is a rare cancer also known as adrenocortical cancer and cancer of the adrenal cortex. It forms in the outer layer of tissue on the adrenal gland, a small organ on top of each kidney that produces steroid hormones, adrenaline and noradrenaline that controls heart rate, blood pressure and other body functions.
Adrenal cortex
In more detail, the adrenal cortex is located on top of each kidney and is the outer portion of the adrenal gland. The adrenal glands are made of two distinct parts; the inner part of the gland, the adrenal medulla, produces nonessential hormones such as adrenaline that helps the body react to stress and the outer part, the adrenal cortex that produces hormones vital to life such as cortisol that regulates metabolism and helps the body respond to stress, and aldosterone which helps control blood pressure. Additionally, the adrenal cortex produces steroid hormones which regulate carbohydrate, fat metabolism and mineralocorticoid hormones which regulate salt and water balance in the body.
The main two groups of corticosteroid hormones that the adrenal cortex releases: glucocorticoids and mineralcorticoids are released when triggered by the hypothalamus and pituitary gland. When the hypothalamus produces corticotrophin-releasing hormone (CRH) that stimulates the pituitary gland to release adrenal corticotrophic hormone (ACTH). These hormones then alert the adrenal glands to produce corticosteroid hormones. Glucocorticoids released by the adrenal cortex include hydrocortisone that regulates how the body converts fats, proteins and carbohydrates to energy, and helps regulate blood pressure and cardiovascular function. Also, it includes the hormone corticosterone that regulates immune response and suppress inflammatory reactions. The mineralcorticoild aldosteroine maintains the right balance of salt and water while helping control blood pressure. The adrenal cortex also releases sex steroids or hormones, however, their impact is usually overshadowed by greater amounts of hormones released by the ovaries or testes.
Adrenocortical Carcinoma
It is a rare cancer, with incidence of one to two million population annually and cases cluster in children under five and in adults thirty to forty years old. Often in adrenocortical carcinoma, it would have invaded nearby tissues or metastasized to distant organs at time of diagnosis so the overall five-year survival rate is only twenty-thirty five percent. As the adrenal cortex makes steroid hormones, benign or cancerous adrenal cortical tumors may affect the rate of production of these hormones, making too much and causing symptoms that affect the body.
People with adrenocortical carcinoma may not have any symptoms until the cancer is quite far advanced and then they may feel pain, tiredness and weight loss. If the tumor does make hormones, this will cause symptoms; it it makes too much hormones like cortisol or aldosterone, symptoms may include raised blood pressure, thirst, passing urine frequently, muscle cramps, weight gain that is more noticeable in the trunk of the body, muscle wasting in legs or arms, fat accumulation in the base of neck, swelling of face to give a moon face appearance, mild diabetes and abnormal hair growth on face, arms and upper back. If the tumor overproduces sex hormones, women may have a deepened voice, changes in periods, baldness or growth of facial hair and men may have loss of sex drive, impotence, and sometimes swelling of the breasts.
Treatment of cortical carcinoma may range from surgery, radiotherapy to chemotherapy or mitotane. However, as adrenal cortical tumors are fast growing, they are often diagnosed in the later stages which means it can be difficult to cure. If the cancer is diagnosed early enough, surgery to remove the affected adrenal gland is usually the first choice of treatment and can cure the cancer. If the cancer has spread locally, surgery would also remove the tissues immediately surrounding the adrenal gland and nearby lymph nodes. If only one adrenal gland is removed, the other would continue making the hormones the patient would need, however, if both are removed, they would have to take hormone replacement tablets for the rest of their lives.
Radiotherapy is used when the cancer has spread and can be useful for shrinking cancer spread to the bones. On the other hand, chemotherapy would use anti cancer drugs to destroy the cancer cells and is usually only used to treat adrenal cortical tumors that have spread to other parts of the body. Some of the chemotherapy drugs used to treat adrenal cortical cancer are: cisplatin, doxorubicin, paclitaxel, fluorouracil, vincristine, etoposide, and mitotane.
