Cancer that starts in the outer layer of the adrenal glands (the adrenal cortex). It often releases hormones that make a person’s breasts or sex organs grow larger, and it can cause other symptoms, such as hair growth on the face or enlarged prostate in men.
The first step in diagnosing ACC is to get a sample of the tumor for study under a microscope. This is called a biopsy.
Adrenocortical carcinoma is rare cancer that starts in the outer layer of the adrenal glands (adrenal cortex). The adrenal glands make hormones, which help control blood pressure and some body features, like hair growth. Benign adrenal tumors often don’t cause symptoms and don’t need treatment. But, sometimes the tumor makes too much of certain hormones and causes problems. Cancer cells can also spread from the adrenal gland to other parts of the body. When this happens, the new tumors are called metastases.
The signs and symptoms of adrenocortical carcinoma depend on what type of hormones the tumor is making. A functioning ACC can make too much androgen or estrogen hormone, which may cause a boy to develop enlarged penis and/or breasts. It can also cause abnormally high blood pressure and an unusually fast heart rate (tachycardia). Nonfunctioning ACCs do not make any hormones and usually don’t cause any symptoms.
Doctors diagnose adrenocortical carcinoma by looking at the patient and doing a physical exam. They will ask about the person’s past health, including any inherited conditions. Then they will do imaging tests, such as a CT scan and an MRI.
CT scans can show the size of the adrenal tumor and any other changes in the structure of the adrenal glands. MRIs can show the size and location of the tumor and whether it is pushing on the kidney or sex organs. They can also help find out if the tumor is spreading outside the adrenal gland.
Blood hormone studies can check for abnormal levels of the hormones testosterone and cortisol. They can also show if the cancer is producing too much of these hormones. The doctors can also do a 24-hour urine test to measure the amounts of the hormones 17-ketosteroids and cortisol. These levels are lower in people with adrenocortical carcinoma than in those who don’t have the disease.
If the adrenocortical carcinoma is in a very advanced stage, it may not be possible to treat it surgically or with medicine alone. When this is the case, doctors give patients a drug called mitotane. This drug slows the growth of the tumor and may keep it from spreading. It can also relieve some of the cancer’s symptoms, such as high blood pressure and nausea.
The adrenal glands are near the front of the kidneys and produce several hormones that control body functions, including blood pressure. Adrenocortical carcinoma (ACC) is a rare cancer that forms in the outer layer of the adrenal glands. The tumor secretes hormones that can cause many different symptoms, including high blood pressure, Cushing syndrome (round face, double chin, weight gain, muscle weakness), and heart attack. ACC can also spread to the liver, lungs, and bones. ACC can occur in adults and children, but it is more common in teenagers and young adults. ACC can be diagnosed by a physical exam and by imaging tests.
Doctors may also ask about your family history of the disease and look for certain signs, such as a lump or mass in your abdomen. They will do a blood test to measure your levels of cortisol and 17-ketosteroids, which are hormones produced by the adrenal glands. These are higher in people with adrenocortical carcinoma. Doctors may also do a biopsy to check for cancer cells. This is done by removing a small sample of tissue, either with a thin needle (fine-needle aspiration or FNA) or using a wider needle (core biopsy). The samples are then studied under a microscope to look for signs of cancer.
In most cases of ACC, the cancer has already spread when it is diagnosed. However, the cancer can be found in asymptomatic people and is often discovered as an incidental finding on an imaging test for another reason (ie, adrenal incidentaloma).
After the diagnosis is made, other tests are done to see whether the cancer has spread within the adrenal gland or to other parts of the body. These include a chest CT scan, an MRI with gadolinium, and a PET scan. Treatment for ACC depends on the stage of the disease, which is determined by looking at how the tumor looks under a microscope and how it responds to treatment. The tumor’s grade determines how likely it is to recur after surgery and what treatments are appropriate. The grading system is based on 3 major and 4 minor criteria.
Adrenocortical carcinoma is very rare — occurring in only about one in 1 million people per year in the United States. It is also more difficult to treat and more likely to be fatal than other adrenal tumors, such as adenomas and pheochromocytoma. There is no known cause of adrenocortical cancer, but it may be caused by a preexisting adenoma that develops into cancer, or it may happen spontaneously. Some cases of adrenocortical carcinoma are linked to hereditary syndromes such as Li-Fraumeni or Lynch syndrome.
The adrenal glands are two small triangular-shaped glands that sit on top of each kidney. They make important hormones that control water balance, blood pressure, stress response, and give the body its male or female traits. Adrenocortical carcinoma occurs in the outer layer of the adrenal glands, called the cortex.
Doctors use imaging tests and blood tests to diagnose adrenocortical carcinoma. They will ask about symptoms and your family history, including a history of hereditary disorders or adrenocortical carcinoma. Your doctor will also feel your abdomen for a mass or lump. Your doctor may also order a genetic test to look for changes in the p53 gene that can lead to adrenocortical carcinoma.
When ACC is diagnosed, your doctor will perform surgery to remove the tumor. If the tumor is not fully removed, it will grow back. You may also need chemotherapy or radiation to reduce the size of the tumor before surgery or to treat the spread of cancer to other parts of your body.
Despite the fact that malignancy is established by the Weiss score, it is important to consider a diagnosis of poorly differentiated adrenocortical carcinoma (PDAC) based on other histopathologic criteria, such as the Helsinki score. The Helsinki score is an alternative to the Weiss score and uses two weighted histological parameters with Ki67, a marker for cell proliferation.
If your doctor recommends surgery, you will be treated by an endocrine surgeon or other surgical oncologist who is familiar with this rare disease. You may also choose to be evaluated for a clinical trial of new treatments at a center that is dedicated to this disease or is part of a national cancer research network.
Adrenocortical carcinoma is a rare tumor that forms in the adrenal cortex. It is usually functioning (makes more hormones than normal), but nonfunctioning adrenocortical carcinomas can also form. These are more likely to be malignant, or cancerous, than adenomas.
ACC occurs most often in adults, but can affect children as well. It is more common in men than in women. The median age at diagnosis is 46 years. It is very important to diagnose and treat adrenocortical carcinoma early, because it can spread rapidly. The prognosis is better for patients who have a low-grade adrenocortical carcinoma and are diagnosed at an early stage, with less aggressive disease.
Doctors diagnose adrenocortical carcinoma by learning about a patient’s symptoms and doing a physical exam. They may also ask about a family history of inherited conditions like Li-Fraumeni syndrome or Lynch syndrome, or previous adrenal tumors. They can then perform tests to check for adrenocortical carcinoma, including blood work and imaging studies.
Treatment options for adrenocortical carcinoma include surgery, chemotherapy, and radiation. They may also recommend other local treatments to help relieve symptoms, such as radiofrequency ablation and cryotherapy. In general, these treatments are used if the cancer is not too large and has not spread to other parts of the body.
Surgery is usually done first, in order to remove the tumor and avoid a more serious problem, such as a ruptured adrenal gland. The next step in treatment is to use chemotherapy to kill any remaining cancer cells. This can be given by mouth or through an IV infusion. Depending on the patient’s health status, doctors can consider using other types of treatment, such as targeted therapy or immunotherapy.
The prognosis for adrenocortical cancer depends on how many lymph nodes are involved and whether the cancer has spread to other parts of the body. It is better to get treatment from specialists who are familiar with this type of cancer. This includes endocrinologists, endocrine oncology specialists, and endocrine surgeons.
A genetic test can be done to see if someone has a gene mutation that increases their risk of getting this cancer. This can be helpful for family members who want to know their own risks of getting the disease.
Doctors may order a blood test that measures cortisol and other hormones. They will also ask about family history of inherited conditions like Li-Fraumeni syndrome or Lynch syndrome that increase the risk of ACC.
A tumor in the adrenal cortex can be functional or nonfunctional. A functioning adrenocortical carcinoma can cause Cushing syndrome and virilization (male characteristics in women). It can also produce the hormones cortisol and aldosterone.
The adrenal glands make hormones that control many body functions, including blood pressure. Cancer of the adrenal cortex occurs when healthy cells mutate and grow out of control. Adrenocortical carcinoma is the most common type of adrenal cancer and affects children and adults. It’s also more common in females than males. Endocrinologist Amir Hamrahian, MD, and endocrine surgeon Lilah Morris-Wiseman, MD, describe the symptoms of adrenocortical carcinoma and discuss how the condition is diagnosed and treated.
The first step in diagnosis is a physical exam and a review of your health history. Your doctor will ask about your past illnesses and family history of disease, especially cancer. Imaging studies may be used to check for a tumor in the adrenal glands or for areas of abnormal tissue growth. These include x-rays, CT scans and magnetic resonance imaging (MRI).
Your doctor will also test your blood to see how much cortisol is in your body. In addition, a 24-hour urine test is done to measure the amount of 17-ketosteroids and other hormones in your urine. A high level of these substances in your urine can be a sign of adrenocortical tumors or a hormone imbalance.
If adrenocortical carcinoma has spread to other parts of the body, more tests will be done to find out how far the tumor has traveled. These may include a blood sample to look for the presence of certain hormones, such as testosterone and estradiol, in your body. A bone scan is also sometimes used to determine if there are any abnormal areas in your bones.
If you have adrenocortical carcinoma that hasn’t spread, surgery is usually the main treatment. Your doctor will remove the adrenal gland, along with a small piece of surrounding tissue. In some cases, when the tumor has spread, chemotherapy or radiation therapy is used with surgery.
If you have adrenocortical carcinoma that has spread to other parts of the body, you’ll need more treatments. These may include chemotherapy, radiation therapy and medicines to reduce your levels of certain hormones. You’ll need regular follow-up with your doctor, which will include exams in the office and blood tests.
Whether the cancer is located in the adrenal cortex or has spread to the kidney, diaphragm, pancreas, or spleen and surrounding blood vessels, it will cause you to gain weight. Often this is due to an excess of hormones produced by the tumor and can be a very difficult symptom to manage. It is important to work with a urologist who has extensive experience with Adrenocortical Carcinoma to help manage your symptoms and ensure the most effective treatment.
The adrenal gland has an outer layer, or cortex, and a center or medulla that makes hormones to regulate the body’s water and salt levels, metabolism, and stress response. In healthy people, tumors that develop in the adrenal cortex are called adenomas. While these growths can be benign, they may produce too many hormones, leading to Cushing’s syndrome, which causes high blood pressure, low potassium and upper body obesity. When the tumors begin to produce too much cortisol and aldosterone, it is referred to as hypercortisolism.
On the other hand, if a tumor grows in the medulla of the adrenal gland, it is considered malignant and called pheochromocytoma. Pheochromocytomas are rare and they usually occur in young people, but they can also be seen in adults. They are thought to develop when cells in the adrenal medulla have genetic mutations that cause them to grow out of control and start making too many adrenal hormones.
Most of the time, a tumor that starts in the adrenal cortex is a primary cancer. However, a tumor that starts in another part of the body and then moves to the adrenal gland is more common. This is called a secondary adrenal cancer and it tends to be more advanced than a primary adrenal tumor.
If your adrenocortical carcinoma has not spread to other parts of the body, it can be treated by surgically removing the adrenal tumor or by using medications to stop the tumor from producing hormones. However, it is not uncommon for adrenocortical carcinoma to recur once the tumor has been removed. It is therefore important to discuss your diagnosis and treatment options with a team of highly trained surgeons, like those at the UCLA Endocrine Surgery team, and with endocrinologists who specialize in adrenal disorders.
A tumor in the adrenal cortex can cause abdominal or flank pain. The pain can be worse at night or when you lie down. Your doctor will ask about your symptoms and do a physical exam. They may also order blood and urine tests to check your levels of hormones. These tests help find out the size of the tumor and whether it’s cancerous or not. They can also show if the tumor is spreading to other parts of the body.
The adrenal glands are two small triangular-shaped glands that sit on top of each kidney. They make important hormones that help control water balance, blood pressure and stress response. Adrenocortical carcinomas form in the adrenal cortex and can be either functioning (producing hormones) or non-functioning (not producing hormones). Functioning ACCs are more common than non-functioning ones. ACCs can be caused by a preexisting adenoma that becomes cancerous, or they can happen sporadically without a known cause.
About 40 percent of people with ACC have no known cause of their cancer. Others have a family history of the disease. This suggests that genetic factors play a role in the development of ACC. It’s also possible that a genetic condition called Li-Fraumeni syndrome increases your risk of developing adrenocortical carcinoma. Other inherited conditions that are linked to adrenocortical carcinoma include multiple endocrine neoplasia and tuberous sclerosis.
Most people with adrenocortical carcinoma that hasn’t spread are treated with surgery to remove the tumor. They may also have chemotherapy.
Radiation therapy is used in a few cases to try to prevent the cancer from coming back after surgery. However, this is not always helpful.
If the adrenocortical carcinoma has spread to other parts of the body, treatment can involve chemotherapy and radiation. You may also need to take long-term medicine to control the amount of hormones in your body. The doses of these medicines change over time. Your doctor will do regular follow-up exams in the office and blood tests to monitor your health. They will also do imaging tests to see if your cancer has returned.
Adrenocortical Carcinoma can cause a great deal of fatigue. The cancer is thought to cause this by causing hormones that affect the body’s metabolism and levels of energy. The fatigue that results from adrenocortical carcinoma is usually not constant and can come and go. It may also occur at different times of the day, depending on what is affecting your hormone levels. The fatigue that results from adrenocortical tumors can be particularly difficult for people who have had surgery to remove the adrenal gland.
If you notice any of the symptoms listed above, see your doctor right away. They will run some tests to determine if the adrenal tumor is causing your symptoms, and they will likely recommend that you receive treatment.
During the physical exam, your doctor will feel your abdomen for any unusual lumps or bumps. They will also take a blood sample to check your cortisol levels, which are indicative of the presence of an adrenal mass. If they are very high, your doctor will want to surgically remove the adrenal gland.
The adrenal glands are two small triangular-shaped glands that sit on top of each kidney. The outer layer of these glands, called the adrenal cortex, produces important hormones that balance salt and water levels in the body, help control blood pressure, regulate metabolism, and help the body respond to stress. Cancers that form in the adrenal cortex are known as adrenocortical carcinoma (ACC).
If your ACC is in its early stages, complete surgical removal can cure it. More advanced tumors may spread to surrounding organs, including the kidney, liver and inferior vena cava. In those cases, you will probably need to have those other tissues removed along with the adrenal gland.
Radiation therapy is a common treatment for adrenocortical carcinoma. It involves a machine outside of the body sending radiation toward the adrenal mass, or sometimes other tissues that have been affected by the cancer. The radiation can be delivered through an external beam or it can be sealed inside needles, seeds or wires that are inserted into the adrenal mass.
Adrenocortical Carcinoma treatment includes surgery, chemotherapy, radiation and other treatments. Our endocrinologists, endocrine oncology specialists and endocrine surgeons are familiar with this rare cancer and have expertise in treating it.
The adrenal glands are two small triangular-shaped glands that sit on top of each kidney. They make hormones that control water balance, blood pressure and stress response. Cancerous cells sometimes form in the outer part of these glands, called the cortex.
The adrenal glands, located on the front of the kidneys, produce hormones that control many body functions, including blood pressure levels and metabolism. Adrenocortical carcinoma is a cancer that forms in the outer layer of the adrenal glands. It usually affects children, but it can occur in adults as well.
The most common treatment for adrenocortical carcinoma is surgery to remove the tumor with the affected adrenal gland (adrenalectomy). Sometimes, nearby lymph nodes and other tissue are also removed. This helps doctors find out if the cancer has spread.
During the operation, surgeons try to leave a margin of healthy tissue around the adrenal gland to improve the chances that all of the cancer will be removed. They may use a minimally invasive procedure, which uses smaller surgical incisions. Alternatively, they may use an open approach, which requires larger surgical cuts.
After surgery, doctors treat you with chemotherapy and hormone treatment. The chemotherapy drugs destroy cancer cells and prevent them from growing. The hormone therapy medicines, which are taken by mouth, help relieve symptoms caused by too many hormones released by the tumor.
You can have these treatments in a doctor’s office, a surgery center, or a hospital. Whether you have outpatient surgery or stay in the hospital depends on the type of surgery and how quickly you recover. You will probably need to fast before your surgery. If you are having outpatient surgery, you will need to bring supplies to care for your wounds at home, such as antiseptic ointment and bandages.
You will need to have preop tests to make sure you are healthy enough for surgery. These might include blood work, chest X-rays, an electrocardiogram (ECG), and a colonoscopy or upper endoscopy. Your practitioner will also order imaging tests, such as a CT scan or an MRI. These tests will be sent to your surgeon before the operation.
For people whose tumors are not surgically removed, chemotherapy can help control the cancer. The goal of treatment is to prevent the tumor from growing again and causing more problems. The chance of this depends on what type of chemotherapy is given, how much is used, and whether it is given in combination with other treatments, such as radiation. The chances of having a good response also depend on how far the cancer has spread when treated with chemotherapy.
The chemotherapy drugs are delivered in ways that will get them into your body where they will have the most effect. This can be done through a needle in a vein (intravenous, or IV) or with a soft, thin tube that goes into a large blood vessel in your neck or chest area (catheter). Drugs are often given at regular intervals called cycles, which include a period of treatment followed by several days or weeks without it. This gives your normal cells time to recover from the effects of the drugs.
Medications used to treat adrenocortical carcinoma are typically given at low doses because high amounts of the medication can cause heart problems, liver damage, and kidney damage. They are given with the help of a specialist nurse or doctor who is familiar with this rare disease.
Because adrenocortical carcinoma is so rare, there is very little information about the best way to treat it. Clinical trials are underway to improve the treatment of this disease.
If you are interested in trying a new therapy, your doctor may refer you to a clinical trial that is accepting patients. You can search for clinical trials using the National Cancer Institute’s cancer trial finder.
Having a rare cancer like adrenocortical carcinoma can be very stressful, especially when you are first diagnosed. It is important to have a team of experts that is familiar with this disease, including endocrinologists, endocrine oncology specialists, and endocrine surgeons. They can give you the best chance of having a successful treatment.
Adrenocortical carcinoma is a rare malignancy originating from the adrenal cortex. It is highly aggressive and typically diagnosed at advanced stage with a poor prognosis. Complete surgical removal remains the only curative treatment. However, two-thirds of the patients present with metastatic disease, frequently involving the liver and lung. Adjuvant radiation can reduce the risk of local recurrence after surgery but it has not been well studied.
The first step in treating ACC is to perform careful endocrine testing to reveal excessive hormone production. This is followed by imaging with computed tomography and/or magnetic resonance imaging of the abdomen. If possible, positron emission tomography can be useful in staging the disease and identifying unsuspected sites of metastases.
In a retrospective study of patients who underwent surgical resection for ACC, the addition of adjuvant radiation significantly improved local recurrence-free survival. The radiation therapy used in this study consisted of conformal three-dimensional radiotherapy delivered over the tumor bed. The patient groups were matched for resection status, use of adjuvant mitotane, clinical stage, and tumor size.
Radiation therapy can be painful and patients may experience side effects such as bruising, nausea, vomiting, fatigue, or hair loss. To minimize these side effects, doctors may administer drugs to help manage the symptoms. In some cases, these drugs may also be given intravenously (through a vein) to prevent allergic reactions to the radiation.
Our team of radiologists, surgeons, and endocrine surgeons will work with your primary care provider to determine the best treatment plan for you. We are dedicated to helping you achieve the best quality of life during and after your adrenocortical cancer journey. We are here to help with any questions or concerns you might have along the way. Contact us today to get started.
Your immune system protects you from diseases by attacking germs and other invaders, including mutated cells that can cause cancer. But cancer cells sometimes hide from the immune system, or trick it into not detecting or attacking them. Immunotherapy helps your body’s natural defences better recognise and destroy cancerous cells.
Immunotherapy has already led to major treatment breakthroughs for many cancers. It’s used alone or in combination with other treatments, like surgery or chemotherapy. Several immunotherapies have been Food and Drug Administration (FDA)-approved to treat cancers of the bladder, head and neck, esophagus, kidney, and lung. And many others are available through clinical trials.
Some types of immunotherapy can trigger flu-like symptoms in some people, including fever, chills, joint and muscle aches, loss of appetite, nausea, vomiting, or fatigue. These side effects are usually mild and go away as your body gets used to the medicine. Taking anti-nausea and anti-fatigue drugs can help reduce these side effects. Your doctor can prescribe them for you.
Immunology is one of the fastest growing areas in cancer research, with new therapies being developed all the time. Scientists are working to find ways to personalise immunotherapy for each patient, based on the unique genetic makeup of their tumour and how it interacts with the patient’s immune system.
Because adrenocortical carcinoma is so rare, it’s important to be seen by experts who are familiar with the disease, such as endocrinologists, endocrine oncology specialists, and endocrine surgeons. Some pheochromocytomas are linked to certain inherited conditions, including neurofibromatosis, von Hippel-Lindau disease, and multiple endocrine neoplasia syndromes such as tuberous sclerosis and Sturge-Weber-Mole-Kirtlin syndrome.
If you’re being treated for adrenocortical carcinoma, our specialists may also recommend genetic tests to look for mutations in your TP53 or IGF2 genes that can cause the tumours to grow more quickly and be harder to treat. We can offer counselling for family members who are concerned about the genetic risks associated with adrenocortical carcinoma. Your healthcare team will give you a number to call at any time of the day or night if you have questions about managing your symptoms.
