Adrenocortical Carcinoma (ACC)

What do the adrenal glands do?

The adrenal glands regulate key body functions through hormone production, including:

• Cortisol – controls stress response and metabolism
• Aldosterone – regulates blood pressure and salt balance
• Androgens and oestrogens – influence sexual development and function

Adrenocortical carcinoma (ACC) originates in the adrenal cortex, whereas phaeochromocytomas arise from the adrenal medulla, the part of the adrenal gland responsible for producing adrenaline and noradrenaline.

When ACC develops, these hormones may be overproduced, leading to a range of symptoms.

Types of ACC

ACC is typically classified into two groups:

Functioning tumours (hormone-producing)

These produce excess hormones and often cause noticeable symptoms early.

Non-functioning tumours

These do not produce hormones and are often diagnosed later, once the tumour grows large enough to cause pain or pressure.

Symptoms of adrenocortical carcinoma

Symptoms vary depending on whether the tumour is producing hormones.

Hormone-related symptoms

• Weight gain, especially in the face and abdomen
• Muscle weakness and fatigue
• High blood pressure
• Acne and skin changes
• Excess hair growth (particularly in women)
• Irregular menstrual cycles or voice deepening
• Breast tissue growth in men

Symptoms from tumour growth (mass effect)

• Abdominal or back pain
• Feeling of fullness
• A noticeable lump in the abdomen

What causes adrenocortical carcinoma?

The exact cause of ACC is often unknown, and most cases occur sporadically.

However, some inherited genetic conditions increase risk, including:

• Li-Fraumeni syndrome
• Beckwith–Wiedemann syndrome
• Lynch syndrome
• Carney complex
• MEN1
• Familial Adenomatous Polyposis (APC)

These conditions affect how cells grow and repair DNA, increasing the likelihood of cancer development.

How is ACC diagnosed?

Diagnosis of ACC involves a combination of hormonal testing and imaging studies. Once ACC is suspected or confirmed, it should be discussed at the time of diagnosis by a multidisciplinary specialist team.

Hormone testing

Blood and urine tests measure hormone levels such as:

• Cortisol
• Aldosterone
• Testosterone or oestrogen

This helps determine whether the tumour is functioning.

Imaging

Scans are used to locate and assess the tumour:

• CT scan (most common)
• MRI
• PET scan (in some cases)

Biopsy

Biopsy is not recommended as part of the investigation of suspected ACC, unless there is evidence of metastatic disease that rules out surgery and histopathological confirmation is required to guide oncology management.

Stages of adrenocortical carcinoma

Staging describes how far the cancer has spread:

• Stage I – small tumour (≤5 cm), confined to the adrenal gland
• Stage II – larger tumour (>5 cm), still confined
• Stage III – spread to nearby lymph nodes or tissues
• Stage IV – spread to distant organs such as the liver or lungs

Staging is critical in guiding treatment decisions and prognosis, and will determine a course of treatment.

Treatment options for ACC

Treatment depends on the stage, hormone activity, and overall health of the patient.

Surgery

Surgical removal of the tumour (adrenalectomy) is the main treatment and offers the best chance of cure when the cancer is localised.

Chemotherapy and radiation

Used in more advanced cases to:

• Slow tumour growth
• Manage symptoms

Mitotane is a specialised drug used to:

• Reduce hormone production
• Lower the risk of recurrence after surgery

Common chemotherapy combinations may include drugs such as cisplatin, etoposide, and doxorubicin.

Prognosis and survival

Outcomes vary depending on how early the cancer is detected.

• Early-stage ACC has significantly better outcomes
• Advanced (stage IV) disease is more difficult to treat

Because ACC is rare and complex, treatment at specialist centres is strongly recommended.

Living with ACC

Managing ACC often involves ongoing care and monitoring.

Hormone management

Treatment can affect hormone levels, sometimes requiring lifelong hormone replacement therapy.

Monitoring and follow-up

Regular scans and blood tests are essential to:

• Detect recurrence
• Monitor treatment response

Side effects and quality of life

Treatments such as mitotane can cause side effects including:

• Fatigue
• Gastrointestinal symptoms
• Cognitive effects

Side effects can differ between individuals and may also be influenced by any additional treatments being received. Close coordination with your care team is important to manage these effectively.

ACC and neuroendocrine cancer

ACC demonstrates important overlaps with NETs:

• Both involve hormone-producing cells
• Symptoms are often driven by hormone excess
• Care is typically delivered by a multidisciplinary team

Patients may benefit from NET-specific support services, particularly when managing complex hormonal symptoms.

Support from NeuroEndocrine Cancer Australia (NECA)

A diagnosis of ACC can be overwhelming, particularly given its rarity.

NeuroEndocrine Cancer Australia provides:

• Access to a specialist NET Nurse, counsellor, and dietitian
• Educational resources and fact sheets
• Guidance on navigating diagnosis and treatment
• Support for patients, carers, and families

Speaking with a specialist nurse can help you better understand your diagnosis and connect with appropriate care.

If you or someone you care for has been diagnosed with adrenocortical carcinoma, it is important to:

• Seek care from experienced specialists
• Understand your treatment options
• Access support early

For more information or support, contact the NECA NET Nurse.