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«University of Michigan Comprehensive Cancer Center Multidisciplinary Adrenal Cancer Clinic ii A Patient’s Guide to Adrenocortical Cancer ©2012 ...»

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Laparascopic surgery. Based on ongoing research done at the University of Michigan, we do not recommended having adrenal cancer (or any mass/nodule suspected of being adrenal cancer) removed laparoscopically. It is thought that laparoscopic instruments rub against the tumor and spread tumor cells to other parts of the abdomen leading to early recurrence. Sometimes the entire tumor is not taken out when the procedure is performed laparoscopically, and the “margins” (edges of the tissue removed) are positive for tumor cells. This leads to recurrent tumor growth and spread in most cases.

An open approach (through a larger incision along the rib cage or up and down the midline of the abdomen) allows the surgeon to remove a margin of normal (benign) tissue around the tumor more easily to help decrease rates of recurrence in the abdomen.

A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center Adrenalectomy The surgical procedure to remove one or both of the adrenal glands is called an adrenalectomy. This procedure can be done in different ways. In general, each of these methods results in the complete removal of the adrenal gland and the entire tumor mass. The surgeon will do a careful inspection of the surrounding organs such as the kidneys, along with all blood vessels, and remove any visible cancer.

Open Adrenalectomy

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The Anterior Approach: the surgeon makes an incision in the abdominal wall (the front of your body). Usually the incision will be horizontal (side to side), just under the rib cage. The incision may carry over to the other side.

Sometimes the surgeon will make a vertical (up and down) incision in the middle of the abdomen. An open anterior approach gives the surgeon the best view of the adrenal glands, surrounding organs and blood vessels. Depending on how aggressive the cancer is, other organs may also need to be removed.

After surgery, patients may spend 3-7 days on average in hospital. Recovery takes four to six weeks after this surgery.

This approach may be combined with an incision into the chest cavity (called the thorax) or through the breast bone (called the sternum). These approaches may be used if the tumor is very large or there is extension of tumor into one of the blood vessels that carries blood back to the heart (called the inferior vena cava).

The Posterior Approach: the surgeon makes a curved incision in the back along the eleventh or twelfth rib. One or two ribs may need to be removed to get to the area of the cancer. This approach is rarely used, but has some advantages, especially in patients who have had extensive abdominal surgery before.

A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center The Retroperitoneal or Flank Approach: the surgeon makes an incision in the patient’s side (called the flank). This approach is rarely used, as it does not allow full inspection of the abdominal cavity to look for evidence of tumor spread, but may be used in patients who have undergone previous extensive intra-abdominal surgery.

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Laparoscopic adrenalectomy is an excellent option for non-cancerous adrenal growths or for cancers that have traveled from another site (metastasized) to the adrenal gland, but is not appropriate for cancers that originate from the

TREATMENT

adrenal gland. This surgery is done using instruments that are inserted through the abdominal wall. The surgeon makes 3-4 small incisions in your abdomen, and instruments are then inserted that allow your surgeon to visualize the area and remove the adrenal gland.

Tumors that are suspicious for, or are known to be cancer, should not be removed using this approach for the reasons stated before. In some cases, your surgeon may decide to start the operation using a laparoscopic approach if the mass appears entirely benign and is being removed due to physical complaints or excess secretion of hormones. If during the operation your surgeon sees anything suspicious, the operation will be converted to an open approach;

however, if the tumor is cancerous, tumor cells may already have been spread throughout the abdomen.

Patients generally stay in the hospital 1-2 days after laparoscopic surgery.

Recovery and returning to normal every-day activities after laparoscopic adrenalectomy is usually 1-2 weeks, with heavy lifting and strenuous physical activity being limited for 4 weeks.

A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center Radiation Therapy Radiation therapy (also called radiotherapy, x-ray therapy, or irradiation) is the use of a beam of energy (called ionizing radiation) to kill cancer cells and shrink tumors. Radiation therapy injures or destroys cells in the area being treated (the target tissue) by damaging their genetic material (DNA), making it impossible for these cells to continue to grow and divide. Although radiation damages both cancer cells and normal cells, most normal cells can recover from the effects of radiation and function properly. The goal of radiation therapy is to damage as many cancer cells as possible, while limiting harm to nearby

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How is Radiation Therapy Used to Treat Adrenal Cancer?





In general, radiation therapy is currently not a standard treatment for

adrenocortical cancer. It can be used for certain circumstances including:

• treatment after surgery to decrease the risk of local recurrence; or • managing or controlling areas of tumor metastasis. This is particularly useful in treating bone involvement of adrenal cancer.

Local recurrence means that cancer cells have come back in the same area of the body where the tumor started and this can happen when tumor cells were left behind or were not killed.

Radiation therapy is usually delivered daily, Monday through Friday for 5 to 7 weeks. The side effects can include fatigue, skin reactions such as redness and irritation, diarrhea and nausea.

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Chemotherapy is the treatment of cancer with drugs that can destroy cancer cells. These drugs often are called anticancer drugs.

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Normal cells grow and die in an orderly way. When cancer occurs, the abnormal tumor cells keep dividing and forming more cells without control. Anticancer drugs destroy cancer cells by stopping them from growing or multiplying.

TREATMENT

Healthy cells can also be harmed, especially those that divide quickly. Harm to healthy cells is what causes side effects. However, healthy cells usually repair themselves after chemotherapy.

Other types of drugs may be used to treat your cancer. These may include certain drugs that can block or increase the effect of your body’s response to the cancer or affect hormone production by the tumor.

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Chemotherapy is the treatment of choice for adrenal cancer that cannot be cured by surgery. This stage of adrenal cancer is usually metastatic (spread to other organs). Chemotherapy drugs can have an effect on the cancer by stopping the growth of cancer cells or their ability to multiply.

The goals of chemotherapy treatment are to control the cancer, keep it from spreading by slowing the cancer’s growth and improve or reduce the symptoms of the disease.

A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center The most common chemotherapy used to treat adrenocortical cancer is Mitotane. This drug has been used the longest in the treatment of adrenal cancer and works by blocking the hormones produced by the cancer, killing adrenal cancer cells.

Mitotane is chemotherapy tablet taken by mouth in several doses throughout the day. It is important to take each dose exactly as scheduled and to have blood levels regularly monitored.

Patients receiving Mitotane therapy are closely supervised by their doctor for common side effects. These may include somnolence or extreme drowsiness

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A number of chemotherapy drugs can be combined either with each other or with mitotane to treat adrenocortical cancer. These include streptozotocin, etoposide, doxorubicin and cisplatin. Two combinations have been studied in the treatment of advanced adrenal cancer: a combination of mitotane and streptozotocin and a regimen called EDP/M (etoposide, doxorubicin and cisplatin and mitotane). A large international phase 3 clinical trial is currently underway to compare these 2 therapies. It is called the FIRM-ACT trial (First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma Treatment). This trial will take several years to enroll the patients needed.

In general, adrenocortical cancer can be aggressive and resistant to most chemotherapy. Most studies have demonstrated response rates of 30% to 50%.

Studies of newer chemotherapy agents are ongoing.

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A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center Clinical Trials Your doctor may suggest that you consider participating in a clinical trial (a research study or protocol) for the treatment of adrenal cancer. Clinical trials are one very important reason that the University of Michigan Comprehensive Cancer Center is able to offer our patients access to the latest cancer treatments.

Clinical trials are used to test new treatments. The goal of these trials is to find ways to improve therapy or decrease side effects. While a trial or study is active or in-progress, we will not know whether any potential improvement has been

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There may be some risks associated with research. Your doctor will discuss both the potential risks and benefits in detail with you and obtain your written permission before starting you on a research protocol.

Oversight committees at the University of Michigan Medical Center conduct an extensive review of all clinical trials. These committees include an “institutional review board” or IRB composed of cancer doctors, doctors in other specialties and lay people. The IRB reviews all protocols before they are available to patients and again at different times during the research to be sure that the protocol remains appropriate and safe for patients.

All patients on a protocol receive the best care possible, and their reactions to the treatment are watched very closely. If the treatment does not seem to be helping, a doctor can take a patient out of a study. Also, the patient may choose to leave the study at any time. If a patient leaves a study for any reason, standard care and treatment will be given.

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The best prognosis for adrenocortical cancers is seen in patients whose cancer is in Stage I or II. This means their cancer is confined to the adrenal gland and can be removed with surgery. The 5 year overall survival rate, which means the number of patients who will be alive 5 years after cancer treatment, is 40-60%.

Stage III cancers have an overall survival rate of 20%. Stage IV cancers overall survival rate is 10% Prognosis is better in younger patients, while hormonesecreting tumors are associated with worse prognosis.

A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center What Care Will I Receive After Treatment?

Patients treated for adrenocortical tumors are usually followed every 3-6 months for several years after treatment.

If your adrenocortical tumor was functional (secreting hormones), your doctor will monitor the levels of these hormones in your blood. Patients treated for a non-functional or non-secreting tumor will have an MRI or CT scan periodically for the first few years.

Your cancer doctor will work closely with your primary care physician to

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If you have cancer, you may notice every ache, pain, or sign of illness. Even little aches may make you worry. The information in this section is designed to help you become an informed partner in your care, but it is only a guide. Self-help can never take the place of professional health care.

Ask your medical team any questions you may have. Also don’t hesitate to tell them about any side effects that you may have. They want and need to know.

Not all patients will experience the symptoms or side effects listed in this section. We include them to help you understand what may be happening to you. We know these are obstacles to your health and quality of life. Your multidisciplinary team will work with you in reducing or eliminating these problems.

Excess Hormone Secretion (Endocrine Syndromes) The adrenal glands secrete a number of essential hormones. Functional tumors

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• Cushing’s Syndrome (too much cortisol) Cushing’s syndrome describes a group of symptoms that are caused by high levels of the steroid hormone, glucocorticoid in the body. Cortisol is a form of glucocorticoid and is produced by the adrenal cortex. High levels of cortisol can be due to problems in a complex system of hormone production that includes not only the adrenal gland, but the pituitary gland and the hypothalamus as well. Symptoms associated with Cushing’s syndrome include a moon face appearance, fat buildup on the trunk of the body,

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A Patient’s Guide to Adrenocortical Cancer ©2012 University of Michigan Comprehensive Cancer Center • Precocious puberty (too much sex steroids) Children with this syndrome will experience puberty earlier than normal due to high levels of sexual steroids.

Adrenal Insufficiency Adrenal insufficiency happens when the body completely lacks adrenal hormones.

Adrenal insufficiency can be caused by several conditions. In adrenal cancer patients it can be caused by the surgical removal of both adrenal glands such as in a bilateral (both sided) adrenalectomy. It can also be caused by some chemotherapies such as mitotane that stop the gland from producing all hormones.

Symptoms of adrenal insufficiency include:



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