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Adrenal Cancer
DESCRIPTION AND TYPES
Adrenal glands are triangular-shaped organs located on top of each kidney. They are primarily responsible for the release of hormones that have a stimulating effect on the heart, blood flow and dilation of arteries.
Each adrenal gland is divided into an outer cortex and an inner medulla. The adrenal cortex produces steroid hormones that maintain blood pressure and salt balance. The adrenal medulla is derived from nerve cells and is a source of catecholamines (adrenaline and noradrenaline), which are “stress hormones” that increase alertness, strength and speed in an emergency.
Adrenal cancer, also known as adrenocortical carcinoma, is a malignant tumor that occurs in the cortex, or outer layer of the adrenal gland. It is a very rare cancer.
About 70% of adrenal cancers are functioning, meaning that they produce hormones, often in excess amounts. Non-functioning tumors do not produce hormones. Unfortunately, only about 30% of these tumors are confined to the adrenal gland at diagnosis, and have spread to the lung, bone, liver or peritoneum (the membrane lining the abdominal cavity).
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PREVENTION
Age is perhaps the biggest risk factor for adrenal cancer. Most cases occur in adults between the ages of 40 and 50, although the disease also strikes children under five years of age.
Although no genetic basis has been determined, people with Li-Fraumeni syndrome may be at risk. Li-Fraumeni syndrome is an inherited mutation of the p53 "tumor suppressor" gene, which can lead to the development of several different types of cancer, including adrenal tumors.
DIAGNOSIS
Symptoms of adrenal cancer vary widely, depending on the hormones produced by the tumor. About 50% of tumors overproduce cortisol, causing symptoms of Cushing's syndrome.
Tumors that produce androgen (the "male" hormone) may cause no symptoms at all in men, but women may experience excess growth of facial and body hair, a deepened voice or irregular menstruation.
Tumors that produce estrogen (the "female" hormone) may cause breast tenderness or enlargement, lowered sex drive and/or erectile dysfunction in men, while women may not notice symptoms until the tumor grows large enough to affect nearby organs.
TREAT
Surgery
Surgery is the frontline treatment for cancers and other diseases of the adrenal gland. Normally, the entire affected adrenal gland is removed (adrenalectomy). If both adrenal glands are removed, patients will have to be on hormone therapy the rest of their lives.
Laparoscopic Adrenalectomy
Some carefully selected patients may be eligible for laparoscopic adrenalectomy, a minimally invasive surgical technique. Three or four one-centimeter incisions are made in the abdomen or the back to insert the laparoscope, which is a camera on the end of a long, slender tube, and tiny instruments that move nearby organs out of the way and excise the diseased adrenal gland. Surgeons watch their progress on video monitors mounted over the operation table. The adrenal gland is cut away from the kidney and placed in a plastic bag for removal through one of the surgical incisions. Patients who are obese or who have had prior abdominal surgery are good candidates for a retroperitoneal procedure, where incisions are made in the back rather than the abdomen.
Laparoscopic adrenalectomy usually takes about one to two hours, but might take longer for complex cases. Patients generally experience shorter hospital stays, less blood loss and shorter recovery times than with standard surgery techniques.
The best candidates for laparoscopic adrenalectomy:
- Have benign tumors
- Are diagnosed with pheochromocytoma, Cushing's Syndrome or adrenal adenomas
- Have hormone overproduction in both adrenal glands (Cushing's disease)
Other candidates for laparoscopy include patients with isolated metastases to the adrenal gland from other sites (lung, breast, and melanoma).
Other Treatments
Patients with suspected adrenocortical carcinoma are best treated with a standard, open surgical procedure to remove the tumor and other affected tissue. For patients with advanced adrenocortical carcinoma, chemotherapy with mitotane may be used to relieve symptoms from progressing disease. This type of cancer is not sensitive to radiation therapy.
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