Jane Doe
What is adrenal cancer?
The adrenal gland has 2 parts. The outer part, called the cortex, is where adrenal cortical tumors start. The cortex makes certain hormones for the body. These hormones are called steroids.
The inner part of the adrenal gland, called the medulla, is really part of the nervous system. Nervous system hormones are made in the medulla. Tumors of the adrenal medulla are not covered here. This information is only about tumors of the adrenal cortex.
Can adrenal cortical cancer be found early?
For some people with adrenal cortical cancer, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.
For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty.
Follow-up care
Follow-up care will be very important after treatment for adrenal cortical carcinoma. One reason for this is that the cancer can come back (recur), even after treatment for early stage disease. You should see your doctor frequently after treatment and less often later on. If you are on treatment with mitotane your follow-up appointments may need to be more frequent. Remember that mitotane will also suppress the usual adrenal steroid hormone production from your other, normal adrenal gland. As a result, you will need to take hormone replacement tablets to protect you against this cortisol deficiency. CT scans may be done periodically to see if the cancer has returned or is continuing to grow. Periodic tests of your blood and urine hormone levels will be done to evaluate the success of medications in suppressing hormone production by the cancer.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.
Seeing a new doctor
some point after your cancer diagnosis and treatment, you may find yourself seeing a new doctor who does not know anything about your medical history. It is important that you be able to give your new doctor the details of your diagnosis and treatment. Make sure you have the following information handy:
The adrenals are small glands that sit above each of the kidneys, as shown in the picture below. The kidneys are found deep inside the upper part of the belly (abdomen).
The adrenal gland has 2 parts. The outer part, called the cortex, is where adrenal cortical tumors start. The cortex makes certain hormones for the body. These hormones are called steroids.
The inner part of the adrenal gland, called the medulla, is really part of the nervous system. Nervous system hormones are made in the medulla. Tumors of the adrenal medulla are not covered here. This information is only about tumors of the adrenal cortex.
What causes adrenal cancer?
Scientists do not know exactly what causes most adrenal cortical tumors. Over the past few years, they have made great progress in learning how certain changes in a person's DNA can cause cells in the adrenal to become cancer. DNA carries the instructions for nearly all the things our cells do. We often look like our parents because they are the source of our DNA. But DNA affects more than the way we look. DNA changes play a part in our risk for getting certain diseases, including some types of cancer. Most DNA changes that are seen in cancers happen during life rather than having been inherited. These changes may be caused by exposure to radiation or cancer-causing chemicals. But most of these changes happen for no known reason.
A risk factor is anything that increases a person's chance of getting a disease. Different cancers have different risk factors. Some risk factors, such as smoking, can be controlled. Others, like a person's age or race, can't be changed. But scientists have found a few risk factors that make a person more likely to get adrenal cancer. But even if a person does have one or more risk factors for adrenal gland tumors, we can't know for sure how large a part they played in causing the cancer.
Family history
Most adrenal cancers are not linked to heredity. But certain clusters of symptoms called syndromes can cause a higher risk of several types of cancer, including adrenal cancer. Some of these syndromes are:
Smoking
Smoking has been suggested as a risk factor by some researchers.
Scientists do not know exactly what causes most adrenal cortical tumors. Over the past few years, they have made great progress in learning how certain changes in a person's DNA can cause cells in the adrenal to become cancer. DNA carries the instructions for nearly all the things our cells do. We often look like our parents because they are the source of our DNA. But DNA affects more than the way we look. DNA changes play a part in our risk for getting certain diseases, including some types of cancer. Most DNA changes that are seen in cancers happen during life rather than having been inherited. These changes may be caused by exposure to radiation or cancer-causing chemicals. But most of these changes happen for no known reason.
A risk factor is anything that increases a person's chance of getting a disease. Different cancers have different risk factors. Some risk factors, such as smoking, can be controlled. Others, like a person's age or race, can't be changed. But scientists have found a few risk factors that make a person more likely to get adrenal cancer. But even if a person does have one or more risk factors for adrenal gland tumors, we can't know for sure how large a part they played in causing the cancer.
Family history
Most adrenal cancers are not linked to heredity. But certain clusters of symptoms called syndromes can cause a higher risk of several types of cancer, including adrenal cancer. Some of these syndromes are:
- Li-Fraumeni syndrome
- Beckwith-Wiedemann syndrome
- Multiple endocrine neoplasia (MEN-1)
- Familial adenomatous polyposis (FAP)
Smoking
Smoking has been suggested as a risk factor by some researchers.
Can adrenal cortical cancer be found early?
It is hard to find adrenal cortical carcinomas early and they are often quite large when diagnosed. Adrenal cortical carcinomas are often found earlier in children than in adults because adrenal cortical cancers in children more commonly secrete hormones. Children will show outward signs of excess hormone production early. For example they may develop very early signs of puberty due to the sex hormones that sometimes are produced by adrenal cortical cancer. In adults, these tumors may be found early by accident, when a CT scan is done for some other health concern.
The American Cancer Society has official recommendations for the early detection of several types of cancer. Because adrenal cancers occur so rarely, the Society does not recommend routine testing for this cancer in people without any symptoms.
Treatments for Adrenal Cancer:
The American Cancer Society has official recommendations for the early detection of several types of cancer. Because adrenal cancers occur so rarely, the Society does not recommend routine testing for this cancer in people without any symptoms.
Treatments for Adrenal Cancer:
Treatment for adrenal cancer depends on the stage of the disease at diagnosis. Options include surgery, chemotherapy, and radiation. Treatment for patients with functioning tumors usually involves using medications to manage symptoms.
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.
Adrenal Cancer Surgery
Surgical removal of the adrenal gland (called adrenalectomy) 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 (e.g., liver, lungs, kidneys) often requires extensive surgery. Adrenal tumors that have not spread are sometimes removed using laparoscopic adrenalectomy, which is performed through a smaller incision.
Chemotherapy to Treat Adrenal Cancer
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. Side effects include gastrointestinal disturbances (e.g., loss of appetite, nausea, vomiting, diarrhea) and neurological disturbances (e.g., depression, lethargy, sleepiness).
When mitotane therapy fails, cisplatin (Platinol®) may be tried, alone or combined with other agents. Drug combinations used include the following:
Neuroblastoma may be treated with chemotherapy in addition to surgery (adjuvant therapy) using carboplatin (Paraplatin®), cyclophosphamide, doxorubicin, and etoposide (Vepesid®).
Side effects of chemotherapy are often severe and include gastrointestinal disturbances, low blood count (anemia), skin disorders, and neurological disorders.
Radiation Therapy to Treat Adrenal Cancer
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.
Other drugs
Other medicines besides mitotane may be used to block hormone production by the cancer. Ketoconazole, aminoglutethimide, and metyrapone can reduce adrenal steroid hormone production. This can help relieve symptoms caused by these hormones, but doesn't shrink the cancer.
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.
Adrenal Cancer Surgery
Surgical removal of the adrenal gland (called adrenalectomy) 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 (e.g., liver, lungs, kidneys) often requires extensive surgery. Adrenal tumors that have not spread are sometimes removed using laparoscopic adrenalectomy, which is performed through a smaller incision.
Chemotherapy to Treat Adrenal Cancer
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. Side effects include gastrointestinal disturbances (e.g., loss of appetite, nausea, vomiting, diarrhea) and neurological disturbances (e.g., depression, lethargy, sleepiness).
When mitotane therapy fails, cisplatin (Platinol®) may be tried, alone or combined with other agents. Drug combinations used include the following:
- Cyclophosphamide (Cytoxin®, Neosar®), doxorubicin (Adriamycin®), cisplatin
- Fluorouracil (Adrucil®, Efudex®), doxorubicin, cisplatin
- Cisplatin with VP-16
Neuroblastoma may be treated with chemotherapy in addition to surgery (adjuvant therapy) using carboplatin (Paraplatin®), cyclophosphamide, doxorubicin, and etoposide (Vepesid®).
Side effects of chemotherapy are often severe and include gastrointestinal disturbances, low blood count (anemia), skin disorders, and neurological disorders.
Radiation Therapy to Treat Adrenal Cancer
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.
Other drugs
Other medicines besides mitotane may be used to block hormone production by the cancer. Ketoconazole, aminoglutethimide, and metyrapone can reduce adrenal steroid hormone production. This can help relieve symptoms caused by these hormones, but doesn't shrink the cancer.
What happens after treatment for adrenal cortical cancer?
For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty.
Follow-up care
Follow-up care will be very important after treatment for adrenal cortical carcinoma. One reason for this is that the cancer can come back (recur), even after treatment for early stage disease. You should see your doctor frequently after treatment and less often later on. If you are on treatment with mitotane your follow-up appointments may need to be more frequent. Remember that mitotane will also suppress the usual adrenal steroid hormone production from your other, normal adrenal gland. As a result, you will need to take hormone replacement tablets to protect you against this cortisol deficiency. CT scans may be done periodically to see if the cancer has returned or is continuing to grow. Periodic tests of your blood and urine hormone levels will be done to evaluate the success of medications in suppressing hormone production by the cancer.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.
Seeing a new doctor
some point after your cancer diagnosis and treatment, you may find yourself seeing a new doctor who does not know anything about your medical history. It is important that you be able to give your new doctor the details of your diagnosis and treatment. Make sure you have the following information handy:
- A copy of your pathology report(s) from any biopsies or surgeries
- If you had surgery, a copy of your operative report(s)
- If you were hospitalized, a copy of the discharge summary that doctors prepare when patients are sent home
- If you received radiation, a copy of your treatment summary
- If you had chemotherapy, a list of your drugs, drug doses, and when you took them
- Copies of your x-rays and scans (these often can be placed on a DVD)