Adrenal Tumor

Overview

Your adrenal glands produce essential hormones like cortisol, adrenaline, and aldosterone that help regulate essential bodily functions. Tumors can develop in the adrenal glands and can be benign (noncancerous) or malignant (cancerous). Most adrenal tumors are benign adenomas, meaning they are noncancerous tumors that do not spread. Most of these tumors do not produce any hormones (nonfunctional), while some can produce abnormal amounts (functional). 

The primary types of adrenal tumors include: 

• Adrenal adenoma: This is the most common type of adrenal tumor, with a tendency to be both noncancerous and slow-growing. Most adrenal adenomas don’t require treatment. 
• Pheochromocytoma: This hormone-secreting adrenal tumor develops in the adrenal medulla, or the innermost part of the adrenal gland, and can produce excess adrenaline. Pheochromocytomas can be either benign or malignant. 
• Adrenocortical carcinoma (ACC): This adrenal tumor is a rare but aggressive form of cancer that originates in the adrenal cortex, or the outermost part of the adrenal gland. It can grow rapidly and can also spread to other organs. 
• Neuroblastoma: This childhood adrenal tumor stems from immature nerve cells in the adrenal medulla. However, it can develop in other nerve tissues throughout the body. Neuroblastoma is the most common type of cancerous tumor in children. 

Each adrenal gland has two parts: the cortex, the outermost part, and the medulla, the innermost part. The cortex produces cortisol, aldosterone, and androgens, and the medulla produces adrenaline. 

• Cortisol regulates blood pressure, blood sugars, and the immune system and causes changes in metabolism to help your body handle stress.
• Aldosterone regulates the blood pressure by controlling the amount of salt in your bloodstream. 
• Adrenal androgens are converted to estrogen and testosterone in other body parts. However, the amount of these hormones is minor compared to the amounts made in both the ovaries and testicles.

The medulla, or inner part of the adrenal gland, produces nervous system hormones like norepinephrine, epinephrine, and dopamine. 

• Norepinephrine responds to stress by increasing heart rate, blood pressure, and alertness through vasoconstriction, or narrowing of the blood vessels.
• Epinephrine, or adrenaline, is the hormone that initiates your body’s “fight or flight” response. It helps your body deploy energy quickly to respond to a perceived threat.
• Dopamine primarily regulates other hormones by interacting with dopamine receptors in the adrenal gland, although the amount is inferior to the dopamine made within the brain.  

What causes an adrenal tumor?

The exact cause of most adrenal tumors is not fully understood, but research points toward a combination of age, genetic, inherited, hormonal, and possibly environmental factors. These include:

• Genetic mutations: DNA changes in adrenal cells can cause uncontrolled growth, leading to the development of benign or malignant tumors. Some of these genetic mutations are sporadic, while others are inherited.
• Inherited genetic syndromes: Adrenal tumors can run in families, especially in the case of inherited genetic conditions like neurofibromatosis type 1, Li-Fraumeni syndrome, von Hippel-Lindau syndrome, Beckwith-Wiedemann syndrome, or multiple endocrine neoplasia (MEN) type 1 and type 2. 
• Hormonal factors: Long-term exposure to excessive hormones like adrenocorticotropic hormone can contribute to adrenal tumor growth. 
• Environmental and lifestyle factors: Possible environmental triggers of adrenal tumors are thought to include prolonged exposure to toxins or carcinogens or radiation exposure, as well as lifestyle and metabolic disorders linked to hormone imbalances, like obesity and type 2 diabetes. 

Adrenal tumors can also form without an apparent cause and are often found incidentally during imaging tests done for a variety of unrelated conditions. These are known as adrenal incidentalomas, and while most are harmless, some can cause hormone imbalances, leading to conditions like Cushing’s disease, Conn’s syndrome, or pheochromocytoma. 

If you or someone you know has a functional adrenal tumor, you might experience a combination of the following symptoms:

• If you have excess cortisol, symptoms can include:
  • Weight gain, especially in the face, neck, and abdomen
  • High blood pressure
  • High blood sugar
  • Thinning skin and easy bruising
  • Mood changes like anxiety or irritability
  • Muscle weakness
  • Osteoporosis
• If you have excess aldosterone, symptoms can include: 
  • High blood pressure that is difficult to control, low potassium levels which can trigger muscle cramps, weakness, frequent urination, and excessive thirst
  • Headaches and fatigue are also commonly experienced due to high blood pressure and low potassium levels
• If you have excess adrenaline or noradrenaline, symptoms can include: 
  • Rapid heartbeat or palpitations
  • Excessive sweating, even when at rest
  • Anxiety, panic attacks, or tremors
  • Sudden and severe high blood pressure
  • Headaches and dizziness

If you or someone you know has a non-functioning adrenal tumor, it is usually discovered on imaging incidentally and will not cause any symptoms. However, if the non-functioning adrenal tumor is large, it can put pressure on nearby organs, which can cause symptoms like abdominal pain or discomfort, back pain, or feeling full or bloated.

Please note that the presence of these symptoms alone doesn’t mean you have an adrenal tumor—a thorough evaluation by a healthcare professional is required to make an accurate diagnosis. Please consult a healthcare professional for an evaluation.

Adrenal Tumor Diagnosis

Diagnosing an adrenal tumor involves imaging studies, hormone testing, and sometimes genetic testing.

Doctors can use the following exams and imaging tests in diagnosing an adrenal tumor:

• Physical exam: First, your healthcare provider will ask about your symptoms, overall health, and family history. Next, they will perform a physical exam to assess neurological function, including reflexes, coordination, strength, and sensation.
• Computed Tomography (CT): A CT scan relies on X-rays to create detailed images of the adrenal gland and help differentiate benign from malignant tumors based on size, density, and appearance.
• Magnetic Resonance Imaging (MRI): An MRI provides detailed images of the adrenal glands and their surrounding structures to determine the size and nature of the tumor. It’s used if a CT scan is inconclusive because it’s more effective at distinguishing soft tissue characteristics of tumors. In the case of pheochromocytomas, an MRI is preferred because the tumor will appear more clearly.
• Positron Emission Tomography (PET) scan: A PET scan evaluates the metabolic activity of the tumor. By highlighting areas with high metabolic activity, the scan can help detect the spread, or metastasis, of adrenal cancer.
• Hormonal testing: Blood and urine tests can detect excessive hormone production. They can include cortisol testing for Cushing’s syndrome, aldosterone and renin levels for Conn’s syndrome, and adrenaline tests for pheochromocytomas.
• Genetic testing: Genetic testing is recommended for those with a family history of adrenal tumors or inherited genetic syndromes. In genetic testing, a blood sample is taken to extract DNA. Typically, genetic testing is most beneficial for those with specific risk factors or family histories.

A combination of these tests and imaging studies can be performed to diagnose a condition and initiate a treatment plan. 

Surgical Treatments

• Adrenalectomy: A surgical procedure in which one or both of your adrenal glands are removed, an adrenalectomy can be performed in two ways: 
  • Laparoscopic adrenalectomy: Used for small, benign adrenal tumors measuring less than 6 cm, this minimally invasive procedure removes the adrenal gland through small, keyhole incisions in your abdomen. This, in turn, means less tissue disruption and much smaller incisions with a faster recovery.  
  • Open adrenalectomy: For suspected cancer, adrenal tumors larger than 6 cm, or tumors invading nearby structures, an open adrenalectomy is done. Unlike its minimally invasive counterpart, this method involves a single, larger incision in the abdomen, side, or back to directly access and remove the adrenal gland. 
• Partial adrenalectomy: In some cases, surgeons will remove your tumor while preserving part of your adrenal gland. Surgeons often use this approach for tumors affecting both adrenal glands, known as bilateral tumors, to prevent adrenal insufficiency.

If one adrenal gland is removed, your other adrenal gland should continue to work normally. In the event both adrenal glands need to be removed, you will need to take medicine to replace the hormones your adrenal glands made. 

Nonsurgical Treatments

Doctors use nonsurgical treatments when surgery for an adrenal tumor isn’t an option due to high surgical risk, an inoperable tumor, or advanced cancer. The following therapies can also be used in conjunction with surgery. 

• Radiation therapy: Radiation is often used when surgery is not an option or is incomplete, and the adrenal tumor continues to grow or secrete hormones despite medication. As a non-invasive treatment, radiation therapy uses precisely aimed beams of radiation to destroy tumors in the body. While it does not remove the tumor, radiation therapy damages the DNA of the tumor cells, which then lose their ability to reproduce and eventually die. It can also be used for pain relief if an adrenal tumor is pressing on surrounding organs. However, the tumor-shrinking effects of radiation therapy may take months or even years to develop.
• Medications: Medications, including ketoconazole, levoketoconazole, or osilodrostat, are helpful in treating cortisol-producing adrenal tumors. Spironolactone can block the effects of excess aldosterone in aldosterone-producing tumors, alpha and beta blockers can help control high blood pressure caused by excess adrenaline in pheochromocytomas, and mitotane can suppress adrenal cancer cells while reducing hormone production.
• Chemotherapy: Chemotherapy is often used to treat adrenal tumors, especially if the tumors have spread or metastasized to other parts of the body and surgery is not an option. You can take chemotherapy drugs orally or intravenously to inhibit the growth of cancer cells, and they can be used in combination with surgery or radiation therapy.
• Targeted therapy: Targeted therapy is a type of cancer treatment that focuses on attacking precise molecules or pathways critical to cancer cells’ growth and survival.
• Immunotherapy: This treatment works via a series of drugs that boost the body’s immune system to recognize and attack the cancer cells.