Adrenal Cancer

Adrenal cancer is a rare cancer that begins as a growth of cells in the adrenal gland.

The adrenal glands are small, triangular glands located on top of each kidney. These glands produce various hormones essential for regulating metabolism, immune response, blood pressure, and other critical functions.

Most adrenal cancers are adrednocortical carcinomas (ACCs). This type of cancer forms in the adrenal cortex. The adrenal cortex is the outer layer of the adrenal gland. It makes steroid hormones that control metabolism, blood pressure, and other bodily functions. Other types of adrenal cancer include neuroblastomas and pheochromocytomas.

Adrenal cancer is rare. It can be an aggressive form of cancer, causing problems throughout the body. However, when caught in its early stages, adrenal cancer can be treatable.

Learn more about adrenal cancer, including its definition, causes, symptoms, diagnosis, treatment, and potential risks and complications.

What is adrenal cancer?

Adrenal cancer is a malignancy that arises from the adrenal gland, leading to abnormal and uncontrolled cell growth. The adrenal glands, located above each kidney, produce various hormones, including cortisol, aldosterone, and adrenal androgens. Adrenal cancer can cause overproduction of these hormones, leading to a range of symptoms, or it can be non-functional and not produce hormones.

Adrenal cancer is considered a type of neuroendocrine cancer. Neuroendocrine tumors (NETs) are rare cancers that develop from hormone-producing cells found throughout the body.

What are the types of adrenal cancer?

Adrenal cancer has three main types. The type of cancer depends on where it develops in the adrenal gland. Adrenal cancer types include:

• Adrenocortical carcinoma (ACC): The most common type of adrenal cancer. ACC forms in the adrenal cortex, which produces steroid hormones that control metabolism and blood pressure, among other bodily functions.
• Neuroblastoma: Neuroblastomas develop in nerve cells throughout the body. Adrenal neuroblastomas form in the inner part of the adrenal gland, called the medulla. These are most common in infants and children.
• Pheochromocytoma: This rare type of tumor develops in the medulla, in cells that produce adrenaline. Most pheochromocytomas are benign, but in rare cases they may be cancerous.

What causes adrenal cancer?

It is not known what causes adrenal cancer.

In most cases, adrenal cancer occurs sporadically without a clear cause. It involves genetic mutations within adrenal cells leading to cancerous growth.

However, adrenal cancer is more common in people who inherit certain conditions that increase their risk factor. Certain hereditary syndromes increase the risk of adrenal cancer. These include:

• Li-Fraumeni syndrome: A hereditary disorder that predisposes individuals to various types of cancer, including adrenal cancer.
• Beckwith-Wiedemann syndrome: A congenital growth disorder that can lead to various tumors, including adrenal cancer.
• Multiple endocrine neoplasia type 1 (MEN 1): A genetic condition associated with tumors of endocrine glands, including the adrenal glands.
• Familial adenomatous polyposis (FAP): A genetic condition involving the development of numerous polyps in the colon and an increased risk of various cancers, including adrenal cancer.

What are the symptoms of adrenal cancer?

Adrenal cancer can cause different symptoms for different people.

Some common signs and symptoms may include:

• Back pain
• Abdominal pain
• Weight loss
• Loss of appetite
• Fatigue

Adrenal cancer can cause hormone changes in the body. Adrenal cancer may produce excess cortisol. These hormonal changes can cause symptoms of Cushing syndrome, including:

• Weight gain (especially around the midsection and upper back)
• Rounded face
• Purple stretch marks
• Muscle weakness
• High blood pressure

The adrenal cancer may produce excess sex hormones (estrogen and testosterone). In females, changes in sex hormones can cause excessive hair growth, deepening of the voice, and irregular menstrual periods. In males, it can cause gynecomastia (breast enlargement), decreased libido, and erectile dysfunction.

In rare cases, the adrenal cancer may produce excessive aldosterone, leading to high blood pressure, low potassium levels, muscle weakness, and frequent urination.

Diagnosis of adrenal cancer

Medical history and physical examination

• Symptom assessment: Detailed inquiry about symptoms such as weight gain, high blood pressure, muscle weakness, and signs of hormone overproduction.
• Medical history: Review of risk factors, including family history of genetic conditions associated with adrenal cancer and previous diagnoses of endocrine disorders.
• Physical examination: Examination focused on identifying signs of hormone overproduction and presence of an abdominal mass.

Diagnostic tests

• Blood tests:
  • Hormone levels: Assessing levels of cortisol, aldosterone, adrenal androgens, and other hormones to detect hormone overproduction.
  • Electrolyte levels: Checking potassium and sodium levels, especially in cases of suspected hyperaldosteronism.
• Urine tests:
  • 24-hour urinary free cortisol test: Measures cortisol levels in urine collected over 24 hours to assess cortisol production.
  • Urinary catecholamines and metanephrines: In rare cases, to rule out pheochromocytoma.
• Imaging studies:
  • CT scan: Detailed imaging to visualize the adrenal glands, identify the presence of a tumor, and assess its size and characteristics.
  • MRI: Further imaging for comprehensive assessment of the tumor and its relationship with surrounding structures.
  • PET scan: Advanced imaging to detect metabolic activity of the tumor and identify potential metastasis.
• Biopsy: In some cases, a fine needle aspiration (FNA) biopsy or core needle biopsy may be performed to obtain tissue samples for histological examination. However, biopsy is typically used with caution to avoid tumor spread.

Treatment of adrenal cancer

Adrenal cancer treatment depends on the size of the tumor and where it is located. Adrenal cancer is more treatable when it is in its early stages and has not spread (metastasized) to other parts of the body.

The main treatment for adrenal cancer is usually surgical removal of the adrenal gland, called adrenalectomy.

In some cases, adrenal cancer treatment may involve radiation therapy, chemotherapy, or other medications. These treatments are often combined with surgery.

Post-treatment care

• Regular follow-up: Monitoring with blood tests to assess hormone levels, imaging studies to detect any recurrence, and overall health assessment. Follow-up is crucial for early detection of recurrence or metastasis.
• Medication management: Hormone replacement therapy may be necessary if both adrenal glands are removed, to maintain normal hormone balance.
• Lifestyle modifications: Dietary adjustments, regular exercise, and management of overall health to support recovery and long-term well-being.

Potential risks and complications

Complications of untreated adrenal cancer

• Metastasis: Spread of cancer to other parts of the body, including the liver, lungs, and lymph nodes, leading to more complex treatment and poorer prognosis.
• End-organ damage: Due to excessive hormone production, leading to conditions such as severe hypertension, diabetes, and osteoporosis.
• Local invasion: Expansion of the tumor into nearby structures, causing pain and dysfunction of adjacent organs.

Long-term considerations

• Ongoing monitoring: Regular follow-up with imaging studies, hormone level assessments, and blood tests to detect any recurrence or metastasis.
• Hormone replacement therapy: Lifelong hormone replacement may be necessary for patients with adrenal insufficiency following treatment.
• Monitoring for recurrence: Continuous surveillance for signs of recurrence or metastasis to ensure timely intervention.

Impact on quality of life

• Symptom relief: Effective treatment can alleviate symptoms, normalize hormone levels, and significantly improve overall well-being and quality of life.
• Informed decision-making: Accurate diagnosis aids in making informed decisions about treatment and preventive measures.
• Emotional support: Providing counseling and support to address anxiety, depression, and the psychological impact of living with adrenal cancer or undergoing treatment.

Adrenal cancer is a rare but aggressive malignancy that requires early diagnosis and appropriate intervention with medical treatments, surgery, or adjunctive therapies to manage symptoms, control hormone levels, and prevent complications. Understanding the causes, symptoms, diagnostic methods, and treatment options is essential for effective management. If you or a loved one are experiencing symptoms or have risk factors for adrenal cancer, consulting with a health care provider, endocrinologist, or specialized surgeon will help determine the need for further evaluation and develop a suitable treatment plan tailored to your specific needs and circumstances.