What Is Adrenal Cancer?

Adrenal cancer is a rare cancer of the adrenal glands. Healthy adrenal gland cells can develop tumours that can become cancerous.¹

Overview

The adrenal glands are endocrine organs that sit atop the kidneys. They are responsible for the production of hormones necessary for normal body functions. Adrenal glands are composed of two parts:

• Cortex - Outer part that makes steroid hormones e.g. cortisol, aldosterone, adrenal androgens
• Medulla - Inner part that makes nervous system hormones e.g. adrenaline, norepinephrine

Tumours can develop in both parts of the adrenal glands leading to adrenal cancer. 

While adrenal cancer isuncommon, there are ways to diagnose and treat it. This article sheds light on potential causes, signs and symptoms, diagnosis and treatment of adrenal cancer, along with other useful information.

Types of adrenal cancer

Adrenal tumours are classified based on tumour location and the ability of the cancer to alter adrenal hormones. Not all adrenal tumours are cancerous.

Tumour location

• Adrenal cortex tumours:
  • Adrenal adenomas – Benign/non-cancerous tumours, often have no symptoms and are found accidentally hence earning the name “adrenal incidentalomas”²
  • Adrenocortical carcinoma (ACC) - Malignant/cancerous tumours¹

• Adrenal medulla tumours:
  • Pheochromocytomas – Rare, benign (10% malignant) tumours
  • Neuroblastomas – arly forms of this cancer can be found in nerve cells and may spread quickly to other parts of the body

Ability to alter adrenal hormones

ACC tumours can be further categorised into:

• Non-functioning tumours: Do not affect hormone levels of the adrenal glands 
• Functioning tumours: Cause the overproduction of adrenal gland hormones²

Stages of adrenal cancer

The stages of adrenal cancer determine the severity of the cancer and the type of treatment needed. Stages can be categorised as early stage or late stage:

Early stage

• Stage 1: Small tumour (≤5 cm) that hasn’t spread to nearby tissues or lymph nodes
• Stage 2: Slightly larger tumour (>5 cm) that hasn’t spread to  nearby tissues or lymph nodes

Advanced stage 

• Stage 3: The tumour is of any size and has spread to nearby lymph nodes, surrounding fatty tissue, nearby organs, or large blood vessels
• Stage 4: The cancer is of any size and has spread to distant organs, bones, or blood vessels¹

Causes of adrenal cancer

The exact cause of adrenal cancer is unknown. Researchers suggest that DNA mutations trigger cancer cell growth. These mutations can arise from:

• Genetic syndromes

While most adrenal cancers are sporadic, some (up to 15%) are caused by inherited genetic defects or syndromes 

• Environmental and lifestyle factors:

There’s no clear evidence that these factors increase the risk of adrenal cancer. However, researchers have suggested that smoking is a possible risk factor¹

Signs and symptoms of adrenal cancer

The symptoms of adrenal cancer occur when adrenal tumours:

• Produce adrenal gland hormones in excess
• Press against other organs as they grow

Signs and symptoms can include:

• Abdominal pain and bloating
• Nausea, vomiting, and fever
• Early onset of puberty
• Excess facial and body hair growth, menstrual irregularities, and balding in people assigned female at birth (AFAB)
• Gynecomastia and erectile dysfunction in people assigned male at birth (AMAB)
• Muscle weakness

Adrenal cancer symptoms are more noticeable in children as they are yet to pass puberty. Howver, they are often only picked up in adults when tumours produce hormones of the opposite sex.¹

Management and treatment for adrenal cancer

Due to the rarity of adrenal gland cancer, research into treatment options is ongoing but slow. At present, the ways to manage and treat adrenal cancer include:^1,2,3,4

• Surgery: The adrenal glands are removed (adrenalectomy). Other organs, tissues, or blood vessels are also removed depending on the extent of tumour spread
• Radiation therapy: High-energy X-rays kill adrenal cancer cells. It’s usually administered after surgery andrecommended only for cases with high chances of recurrence 
• Chemotherapy: Mitotane is the drug of choice to manage adrenal cancer. It kills adrenal cancer cells (and healthy cells) and halts hormone production 
• Hormone therapy: It involves administering medicines that prevent the complications of the overproduction of adrenal gland hormones

Diagnosis of adrenal cancer

The tests used to diagnose adrenal cancer include:^1,2,4

• Physical exam and medical history: Medical professionals examine patients’ abdomens for tumours and ask about their symptoms and family’s medical history 

• Imaging tests: Medical professionals locate adrenal masses and check if adrenal tumours have spread to nearby organs. They include MRI, PET, CT scans, X-rays, and ultrasounds

• Urine and blood tests: These tests can detect high levels of adrenal hormones, even before symptoms appear

• Laparoscopy: Also known as keyhole surgery, a laparoscope is used tolocate adrenal tumours to checks if they’ve spread and surgically remove smaller tumours. 

• Adrenal gland analysis: If a patient’s adrenal glands are removed and sent to a lab, a pathologist studies them to check for cancer and the type of cells affected.^1,2,4

As adrenal cancers are so rare, the American Cancer Society does not recommend regular testing for people with no symptoms.

Risk factors

People with the following inherited genetic conditions are at a higher risk of developing adrenocortical carcinoma:^1,3

• Beckwith-Wiedemann syndrome
• Carney complex.
• Familial adenomatous polyposis (FAP)
• Li-Fraumeni syndrome
• Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC)
• Multiple endocrine neoplasia (MEN1)
• Neurofibromatosis Type 1 (NF1)
• Von Hippel-Lindau (VHL) syndrome

In about 50% of adrenocortical carcinoma cases, the disease runs in families.

Complications

Without treatment, adrenal tumours can grow and cause complications. Non-functional tumours may press against other organs affecting their function, while functional tumours may overproduce hormones and cause conditions such as:

• Cushing syndrome - Caused by the excess production of cortisol, a hormone that helps the body respond to stress. Some symptoms of this disease include:
  • High blood pressure and blood sugar
  • Weight gain in the face, the trunk, and above the collarbone
  • Fat deposition on the neck and shoulder blades
  • Excessive body hair growth and menstrual problems in people AFAB

• Hyperaldosteronism/ Conn’s syndrome - Caused by high levels of aldosterone, a hormone that regulates blood pressure and helps the kidneys control sodium and potassium levels in the blood. The symptoms of this disease include:
  • High blood pressure
  • Muscle cramps and weakness
  • Excessive thirst and frequent urge to urinate
    
• Oestrogen and testosterone imbalance - The adrenal glands produce adrenal androgens, the hormonal precursors of oestrogen and testosterone. Excess production of these sex hormones causes changes in the body.

• High levels of oestrogen can cause:
  • Weight gain, menstrual irregularities, and menopausal problems in people AFAB
  • Impotence, loss of sex drive, and breast enlargement in people AMAB 

• High levels of testosterone can cause:
  • Excessive body and facial growth, irregular periods, and balding in people AFAB^1,2

FAQs

How can I prevent adrenal cancer

Adrenal cancer cannot be prevented as it has no preventable risk factors. People with a family history of genetic syndrome should consult their doctor about regular screening to detect it early. 

How common is adrenal cancer

Adrenocortical carcinoma is very rare. It has a yearly worldwide incidence of 0.5 - 2 cases per million people. In the UK, around 220 people are diagnosed with it yearly. It is more common in people AFAB than in people AMAB (1.5:1 ratio). It can occur at any age but peaks in adults aged 40-50.^3,4

When should I see a doctor

You should contact your doctor if you are experiencing:

• Symptoms of hormonal imbalance
• Abdominal pain and bloating
• Unusual weight gain or loss

Summary

Adrenal cancer is the formation of tumours in the adrenal glands. It’s a rare cancer with no specific cause. However, having genetic syndromes increases its risk. Adrenal cancer care mainly involves surgery and preventing recurrence. Always contact your healthcare provider if you notice any irregualr symptoms as leaving adrenal cancer untreated could result in serious complications.