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Adrenal Gland Cancer is a rare but extremely serious disease that develops in the adrenal glands, which manage the hormonal balance of the human body. This type of cancer, which usually progresses slowly, is often noticed incidentally because it does not show clear symptoms in the early period. Since the adrenal glands play a role in vital functions such as stress management, blood pressure control, and metabolic balance, tumors that develop in this region can directly affect many systems of the body. As the disease progresses, various problems such as hormonal imbalances, weight changes, high blood pressure, and fatigue may occur. This type of cancer, which can be controlled with early diagnosis and proper treatment, can show a high treatment success rate through regular check-ups and specialist follow-up.
What Is Adrenal Gland Cancer?
Adrenal gland cancer, medically known as adrenocortical carcinoma, is a rare type of cancer that occurs through the uncontrolled proliferation of cells in the adrenal glands, which are located just above the kidneys and are responsible for hormone production. Every individual has two adrenal glands, and these glands play a vital role in maintaining the body’s hormonal balance. Hormones such as adrenaline, cortisol, and aldosterone are produced in these glands and are effective in many processes such as the body’s stress response, blood pressure control, and metabolic balance.
Adrenal gland cancer is generally known as an aggressive tumor. It is quite difficult to detect in the early stage because it may not cause symptoms in the initial periods. It is often noticed during imaging tests performed incidentally. As the cancer progresses, symptoms related to hormonal imbalances and organ pressure appear.
Adrenocortical carcinoma can be seen in children and adults, but it is most commonly diagnosed in individuals between the ages of 40 and 50. Its slightly higher incidence in women compared to men suggests the effect of hormonal factors. The tumor usually develops in a single adrenal gland, but in the case of metastasis, it may spread to other organs.
What Causes Adrenal Gland Cancer?
The exact cause of adrenal gland cancer is not fully known, but it has been found to be associated with genetic mutations and some hereditary syndromes. DNA damage that leads to uncontrolled cell proliferation forms the basis of this type of cancer. Genetic disorders, especially hereditary conditions such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and Lynch syndrome, may increase the risk of developing adrenocortical carcinoma in an individual.
In addition, some environmental factors may also be effective in the formation of adrenal gland cancer. Long-term exposure to toxic substances, radiation, smoking, and excessive contact with certain chemicals increase the risk of cell mutation. In addition, hormonal imbalances, especially conditions such as excessive cortisol production in the body, may prepare a suitable ground for cancer development.
Some scientific studies have shown that severe obesity and long-term stress may also indirectly cause structural changes in the adrenal glands. However, these factors are considered supportive risk factors rather than direct causes of cancer. As a result, adrenal gland cancer usually occurs through the combination of multiple factors.
What Are the Symptoms of Adrenal Gland Cancer?
Symptoms of adrenal gland cancer vary depending on which hormones the tumor produces excessively. If the tumor produces hormones, it is called a “functional tumor”; if it does not, it is called a “non-functional tumor.” Functional tumors can usually be noticed early because they cause significant hormonal disorders. Non-functional tumors, on the other hand, may remain silent for a long time.
Among the most common symptoms are Cushing syndrome-like symptoms such as excessive weight gain, rounding of the face (moon face), muscle weakness, high blood pressure, excessive hair growth, bruising and thinning of the skin. These symptoms are caused by excessive production of the cortisol hormone. In women, signs of androgen excess such as deepening of the voice, menstrual irregularity, and acne on the face may also be seen.
As the tumor grows, a feeling of fullness in the abdominal area, pain, loss of appetite, and fatigue may occur. In advanced stages, symptoms related to the relevant organs appear as a result of spread to the liver, lungs, or bones. For example, jaundice or nausea may be seen in liver metastasis, while shortness of breath may be seen in lung metastasis. Therefore, symptoms in adrenal gland cancer follow a very wide spectrum and may be confused with other diseases.
What Are the Risk Factors for Adrenal Gland Cancer?
Some risk factors that increase the likelihood of developing adrenal gland cancer have been scientifically defined. Genetic predisposition comes first among these. The risk is significantly higher in individuals with a family history of adrenocortical carcinoma or hereditary cancer syndromes. Especially in individuals carrying Li-Fraumeni syndrome, such cancers may appear at a young age.
Age is also an important factor. In middle-aged and older individuals, weakening of cell renewal mechanisms facilitates mutation accumulation. In addition, due to hormonal fluctuations in women, the risk is slightly higher compared to men.
Factors such as exposure to radiation, long-term contact with chemical solvents, weakness of the immune system, and chronic stress may also indirectly affect the health of the adrenal glands. In addition, some studies show that long-term hormone treatments (for example, corticosteroid use) may cause structural deterioration in gland tissues.
Although these risk factors do not guarantee the development of the disease, regular medical check-ups are critically important for early diagnosis in individuals who have these factors.
Is Adrenal Gland Cancer Dangerous?
Adrenal gland cancer is a serious disease despite being rare. Its danger generally stems from late detection and aggressive progression. These tumors may grow rapidly and metastasize to surrounding tissues and distant organs. If not diagnosed at an early stage, the chance of surgical intervention decreases and the disease becomes difficult to control.
Since it directly affects hormone production in the body, this type of cancer may cause not only organ damage but also systemic disorders. For example, excessive cortisol production may lead to high blood pressure, diabetes, muscle wasting, and decreased immunity. This condition negatively affects the course of the disease.
However, long-term survival rates are quite high in tumors detected at an early stage and surgically removed. Today, thanks to advanced imaging techniques, minimally invasive surgical methods, and targeted therapies, adrenal gland cancer has become a disease that can be controlled.
What Are the Types of Adrenal Gland Cancer?
Adrenal gland cancer is divided into two main types according to the tissue from which it originates and whether it produces hormones: adrenocortical carcinoma and pheochromocytoma. There are significant differences between these two types in terms of cellular structure, behavior, and response to treatment.
Adrenocortical carcinoma occurs in the cortex layer, that is, the outer part of the adrenal gland. This layer is responsible for the production of hormones such as cortisol, aldosterone, and androgens. Therefore, a tumor developing in this region usually disrupts hormonal balance. Patients most often present with symptoms such as Cushing syndrome, high blood pressure, or virilization (male-pattern hair growth and deepening of the voice in women). Adrenocortical carcinoma is generally a rapidly growing tumor that can spread to surrounding tissues and has a high tendency to metastasize.
Pheochromocytoma, on the other hand, occurs in the medulla layer, that is, the inner part of the adrenal gland. This region is where adrenaline and noradrenaline hormones are produced. Although pheochromocytomas are generally benign, in some cases they may be malignant, meaning cancerous. Depending on hormone secretion, it may manifest with symptoms such as sudden blood pressure elevation, heart palpitations, excessive sweating, and headache. Pheochromocytoma can usually be completely treated when surgically removed.
Apart from these, there is another type known as adrenal metastases. In this condition, the cancer does not start in the adrenal gland; it reaches this region by spreading from another organ (for example, lung, breast, kidney, or colon). Since the adrenal glands have rich blood circulation, they are quite open to metastases. In such cases, the main treatment is aimed at controlling the primary tumor.
Finally, adrenal incidentalomas, meaning small masses detected incidentally, are also noteworthy. Most of these are benign, but some may turn into cancer over time. Therefore, regular follow-up of incidental adrenal tumors is vital.
How Is Adrenal Gland Cancer Diagnosed?
Diagnosis of adrenal gland cancer is usually made through a combination of a detailed medical history, physical examination, laboratory tests, and advanced imaging methods. Since this disease may show symptoms similar to many other endocrine disorders, diagnosis requires great care and expertise.
In the first stage, the doctor listens to the patient’s symptoms and requests blood and urine tests to measure hormone levels. Thanks to these tests, cortisol, aldosterone, androgen, and catecholamine levels are evaluated. Higher-than-normal hormone levels may indicate that the tumor is a hormone-producing type.
Then, the structure of the adrenal gland is examined in detail with computed tomography (CT) and magnetic resonance imaging (MRI) methods. These imaging techniques show the size, location, and relationship of the tumor with surrounding tissues. Positron emission tomography (PET) scans are highly effective in evaluating whether the tumor has metastasized to other organs.
In some cases, biopsy may be performed to make a definitive diagnosis. However, biopsy is not always preferred in tumors in the adrenal region because hormone release may increase during the procedure and cause serious complications. Therefore, the biopsy decision is made only through the joint evaluation of experienced oncologists and endocrinologists.
As a result of all these tests, the stage of the disease is determined. Staging is a critical step in treatment planning. While the tumor is localized in stages I and II, spread to surrounding tissues or distant organs is present in stages III and IV. Diagnosis at an early stage greatly increases the possibility of completely treating the disease.
Adrenal Gland Cancer Treatment
Adrenal gland cancer treatment is personalized by considering the type and stage of the tumor, hormone production status, and the patient’s general health conditions. The most effective treatment method is usually complete removal of the tumor through surgical intervention. Surgical treatment provides a high success rate especially in adrenocortical carcinomas detected at an early stage.
Surgery is usually performed with the laparoscopic (closed) method. However, if the tumor is large or has spread to surrounding tissues, open surgery is preferred. Patients are closely monitored after surgery because there is a risk of recurrence. If necessary, chemotherapy or radiotherapy may be applied after the operation.
In advanced-stage cases, a special drug called mitotane is used in addition to surgery. Mitotane targets adrenal cortex cells, suppresses hormone production, and slows the growth of tumor cells. This drug is sometimes used alone and sometimes together with other chemotherapeutic agents.
In recent years, immunotherapy and targeted therapy methods have also shown promising results in the treatment of adrenal gland cancer. In these methods, special molecules on the surface of tumor cells are targeted, preventing damage to healthy cells. Thus, treatment effectiveness increases and side effects decrease.
The treatment process aims not only to eliminate cancer but also to restore hormonal balance. Therefore, patients may receive hormone replacement therapy under the supervision of an endocrinology specialist. If both glands are removed surgically, lifelong hormone therapy is mandatory.
Can a Person Live Without Adrenal Glands?
It is possible to live without adrenal glands, but in this case, lifelong medical support is required because the body’s hormone production will completely stop. The adrenal glands play a critical role in the stress response, blood pressure control, blood sugar balance, and metabolic processes. Without these glands, the body cannot establish balance on its own.
If both glands are surgically removed, lifelong hormone replacement therapy is applied to the patient. Hydrocortisone or prednisolone is prescribed to correct cortisol deficiency, and fludrocortisone is prescribed to balance aldosterone deficiency. If these medications are not taken regularly, a serious condition called Addisonian crisis may develop. This crisis may result in sudden blood pressure drop, loss of consciousness, and organ failure.
Therefore, life without adrenal glands is possible, but careful medical follow-up and meticulous medication use are essential. Patients should make dose adjustments in stressful situations and always carry an emergency card with them. With appropriate treatment, these individuals can live a long and healthy life.
Why Is Early Diagnosis Important in Adrenal Gland Cancer?
Early diagnosis is one of the most important factors in extending survival and increasing the chance of full recovery in adrenal gland cancer. Because this disease usually progresses silently and often reaches an advanced stage by the time it causes symptoms. Tumors detected at an early stage can be completely removed surgically, and the risk of metastasis can be minimized.
Another advantage of early diagnosis is that hormonal imbalances can be noticed and controlled in time. For example, if cortisol excess is detected early, complications such as hypertension, diabetes, and osteoporosis can be prevented before they develop.
Today, thanks to advanced imaging methods and hormone tests, adrenal gland tumors can be caught at an early stage even incidentally. Therefore, it is of great importance for individuals with genetic risk to undergo regular screenings.
What Happens If My Adrenal Gland Cancer Recurs or Spreads?
Adrenal gland cancer is a disease with the potential to recur after treatment. Especially if the tumor is large, has spread to surrounding tissues, or could not be completely removed during surgery, the risk of recurrence increases. In case of cancer recurrence, metastases are usually seen in organs such as the liver, lungs, or bones.
In this case, treatment is replanned. Surgery may be performed again in suitable patients, but systemic treatments usually come to the forefront. Mitotane treatment, chemotherapy, and immunotherapy combinations can control the spread of the disease. In addition, pain control and supportive treatments are applied to improve patients’ quality of life.
In widespread disease, the aim is to extend survival and preserve quality of life rather than completely eliminate the cancer. Regular follow-ups, hormone level controls, and imaging tests are very important in this process. With appropriate treatment protocols, many patients may experience long-term remission periods.
Adrenal Gland Cancer is a rare disease that seriously affects the body’s hormonal balance and requires careful diagnosis and treatment. Thanks to early diagnosis, the correct treatment strategy, and regular medical follow-up, a large portion of patients can maintain a healthy life. It is critically important especially for people in the risk group not to neglect routine check-ups in order to detect the disease at an early stage.
Prof. Dr. Berkan Reşorlu, born in Ankara in 1979, completed his medical education at Ankara University between 1997 and 2003, and his specialization in urology at Ankara University Faculty of Medicine between 2003 and 2008. Academically, he received the title of associate professor in 2013 and professor in 2022.
He has conducted extensive case series studies in the field of endoscopic stone surgery, contributed surgeries performed on children under the age of 1 to the literature, and has gained international recognition with his stone scoring system and experimental surgical models.
