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Adrenal Tumors

Adrenal tumors are extremely rare with only 150-300 new cases per year. The adrenal glands are located on top of the kidneys, consisting of two parts…the inner area, called the medulla, and the outer area, called the cortex. The job of the adrenal glands is to produce hormones:

The cortex produces three major hormones:

  • cortisol (a glucocorticoid),

  • aldosterone (a mineralocorticoid),

  • dehydroepiandrosterone (DHEA; an androgen).

The medulla produces:

  • epinephrine (adrenaline),

  • norepinephrine,

  • dopamine.

The tumors may or may not secrete hormones, which can cause significant symptoms. The Cushings syndrome results in a swollen face, obesity, high-blood pressure, sugar metabolism problems, and nerve problems. Other tumors may cause hair growth (male patterns) in women or breast swelling in men. The most common type of adrenal cancer is adrenocortical carcinoma. Some cancers, such as breast cancer or lung cancer may metastize to the adrenal glands, but are still treated as the "primary cancer"…breast or lung. Cancers that develop in the adrenal medulla include neuroblastoma (originates in undeveloped nerve cells) and pheochromocytoma (originates in cells that produce epinephrine and norephinephrine). Neuroblastoma usually occurs in infants and children and is covered in its own chapter herein. Pheochromocytoma more commonly occurs in people who are in the 30 to 40 year old group.

Pheochromocytomas require treatment before surgery (neoadjuvant treatment) for high blood pressure, which often includes alpha-blockers (e.g., phenoxybenzamine, prazosin) followed by beta-blockers (e.g., propranolol), and metyrosine.

Testing for pheochromocytomas are as follows:

24-hour urinary catacholamines and metanephrines: This study is designed to measure production of the different types of adrenaline compounds that the adrenal makes. Since the body gets rid of these hormones in the urine, the patient's urine is collected for 24 hours to determine if there is an over-production in adrenaline compounds. This test measures different types of adrenaline (epinephrine, norepinephrine, dopamine) as well as the break-down products of these compounds which the liver and kidney have degraded. Since these compounds are concentrated in the urine, this test is very good at making the diagnosis of pheochromocytoma.

Serum catacholamines (Blood Test): This study measures adrenaline compounds in the blood. It is not as sensitive a test for pheochromocytoma as the 24-hour urine test (sometimes the urine test will be positive and the blood test will be negative), but it still can give important information if it shows elevated adrenaline levels.

X-Ray Tests for Pheochromocytomas: There are 4 primary x-ray tests to examine the adrenal glands (and the rest of the abdomen) for the presence of a pheochromocytomas.

Surgical Treatment of Pheochromocytomas:

All pheochromocytomas should be removed surgically. The vast majority of patients can be treated with the new technique of minimally invasive Laparoscopic Adrenalectomy. This is now the preferred method for removing pheochromocytomas and is available in most hospitals in the U.S.

Adrenalectomy, the surgical removal of the adrenal gland, is the only cure for adrenal cancer. It is important to determine if the cancer has spread before surgery, because metastases to lymph nodes or other organs (liver, lungs, kidneys) often requires extensive surgery. Large adrenal tumors that have not spread are sometimes removed using general surgical procedures.

Chemotherapy is a treatment that travels throughout the body via the bloodstream (called a systemic treatment) that often uses a combination of drugs to destroy cancer cells. It is used as a palliative treatment for metastatic adrenal cancer and may also be used in addition to surgery (adjuvant therapy). Drugs may be administered orally or through a vein (intravenously).

Mitotane (Lysodren®) suppresses adrenal gland function and is the drug of choice to treat inoperable adrenal cancer. Approximately 20% of adrenal cancer patients respond to treatment with mitotane, though side effects, which include gastrointestinal disturbances (loss of appetite, nausea, vomiting, diarrhea) and neurological disturbances (depression, lethargy, sleepiness), can be severe. When mitotane therapy fails, cisplatin (Platinol®) may be tried, alone or combined with other agents such as cyclophosphamide (Cytoxin®, Neosar®), doxorubicin (Adriamycin®), cisplatin, fluorouracil (Adrucil®, Efudex®), doxorubicin, cisplatin® Cisplatin with VP-16

Radiation Therapy uses high energy x-rays to destroy cancer cells. Radiation is not used as a primary treatment for adrenal cancer. It is sometimes used as a pain relieving (palliative) treatment for metastatic adrenal cancer.

Medical Management for patients with functioning tumors includes managing symptoms caused by increased hormone production. Increased cortisol production, know as Cushing's syndrome, is often treated with aminoglutethimide or ketoconazole (Nizoral®) to inhibit cortisol build-up (synthesis). They may be used alone, or in combination with chemotherapy. Side effects include nausea, vomiting, and abdominal pain.

Excess aldosterone production, called Conn's syndrome, is usually treated using spironolactone (Aldactone®). Spironolactone is an aldosterone antagonist (it counteracts the action of aldosterone). Side effects include ulcers, abnormal breast enlargement in men (gynecomastia), fever, and headache.

Aromatase inhibitors such as anastrozole (Arimidex®) and anti-androgens such as bicalutamide (Casodex®) may be used to treat excessive androgen production.

Pheochromocytoma:

Pheocyhromocytomas are rare tumors of the adrenal glands that may secrete catacholamine hormones including norepinephrine and/or adrenaline. These tumors may produce high-blood pressure, headaches, nausea, sweating, anxiety, and tremors

Pheochromocytoma, a rare cancer, is a disease in which cancer (malignant) cells are found in special cells in the body called chromaffin cells. Most pheochromocytomas start inside the adrenal gland (the adrenal medulla) where most chromaffin cells are located. There are two adrenal glands, one above each kidney in the back of the upper abdomen. Cells in the adrenal glands make important these important hormones to help the body function correctly. Usually pheochromocytoma affects only one adrenal gland. Pheochromocytoma may also start in other parts of the body, such as the area around the heart or bladder. Most tumors which start in the chromaffin cells are benign tumors, and do not spread to other parts of the body and are not cancer. If a tumor is found, the doctor will need to determine whether it is cancer or benign.

Pheochromocytomas often cause the adrenal glands to make too many hormones called catecholamines. The extra catecholamines cause the high blood pressure (hypertension), which can cause headaches, sweating, pounding of the heart, pain in the chest, and a feeling of anxiety. High blood pressure that goes on for a long time without treatment can lead to heart disease, stroke, and other major health problems.

Diagnosis and prognosis can vary with each patient. Pheochromocytoma is sometimes part of a condition called multiple endocrine neoplasia syndrome (MEN). People with MEN often have other cancers (such as thyroid cancer) and other hormonal problems. The chance of recovery depends on how far the cancer has spread, and the patient's age and general health. If there are symptoms, a doctor may order blood and urine tests to see if there are extra hormones in the body. The doctor may use a CT scan, or an MRI scan, to make a picture of the abdomen and other internal organs, which may be helpful in making a diagnosis.

Surgery is the most common treatment of pheochromocytoma. A doctor may remove one or both adrenal glands in an operation called adrenalectomy. The doctor will look inside the abdomen to make sure all the cancer is removed. If the cancer has spread, lymph nodes or other tissues may also be taken out.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.
Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation comes from a machine outside the body (external radiation therapy).

The newest clinical trial for pheochromocytoma will evaluate the effectiveness of Iodine 131MIBG (I-131MIBG) in treating malignant pheochromocytoma and whether sensitization medications improve the response to treatment. I-131 MIBG is metaiodobenzylguanidine, a taget agent fused with iodine 131. Pheochromocytoma is a rare type of tumor that usually occurs in the adrenal glands. The tumor cells release chemicals like adrenaline that can cause large increases in blood pressure and pulse rate, with serious health consequences. Tumor in the adrenal glands usually can be removed surgically, but if the pheochromocytoma has spread to many sites in the body, or is located in places where surgery is difficult or impossible, no satisfactory treatment is available. I-131MIBG is a combination of an adrenaline-like chemical and a radioactive form of iodine. The I-131MIBG attaches to the tumor cells and the high concentration of radioactive iodine kills them. Previous studies using 131MIBG to treat pheochromocytoma had a 36% response rate in terms of complete or partial improvement. This study will examine whether adding other sensitization medications to the 131MIBG treatment regimen will enhance its effectiveness in reducing the size and number of tumors.

 

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Last modified: Friday November 28, 2008.