Sasha, VIC
- Adrenocortical carcinoma
August 18th, 2008 is a day I will never forget. A day that will forever be burned into my mind. The day I first heard the words, “Sasha, it’s cancer.”
Adrenocortical Carcinoma (ACC)
What is adrenocortical carcinoma?
Adrenocortical carcinoma (ACC) is a rare and aggressive cancer that develops in the outer layer (cortex) of the adrenal glands, which sit above the kidneys and produce essential hormones.
ACC affects around 1 to 2 people per million each year worldwide, making it one of the rarer endocrine cancers.
Some tumours produce excess hormones, while others grow silently until they become large enough to cause symptoms.
Although ACC differs from the more commonly recognised neuroendocrine cancers (NETs), it shares important neuroendocrine and endocrine features through its role in hormone production. This means many aspects of diagnosis, treatment, and supportive care overlap with those of NETs.
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.
Frequently asked questions about ACC
By definition ACC is a rare cancer which can occur due to an inherited risk of genetic mutations. Conditions which can predispose to ACC are types of NETs such as Multiple Endocrine Neoplasia (MEN1), Neurofibromatosis Type 1 (NF1), Von Hippel Lindau (VHL) syndrome.
Yes. ACC is considered an aggressive cancer, particularly if diagnosed at a later stage. If you begin to show symptoms, see your GP.
Surgery is the primary treatment for localised ACC. Additional therapies such as mitotane, chemotherapy or radiation therapy may be used depending on the stage.
If detected early and completely removed with surgery, ACC can sometimes be cured. Advanced cases are usually managed rather than cured.
Common symptoms include unexplained weight gain, hormonal changes, high blood pressure, and abdominal pain.
Next steps
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.
Helpful Resources
Fact Sheets
5HIAA
Causes & Risk Factors
Abdominal Pain in NETs
Booklets & Guides
About NETs
Booklets & Guides
Acronyms & Terminology for Neuroendocrine Cancer Patients
Causes & Risk Factors
Age and Gender as Risk Factors for NETs
Booklets & Guides
Associated Conditions
Clinical Trials
AUS-NET – Neuroendocrine Tumours
To improve patient quality of life and develop a cost-effective system that can be implemented across other rare cancers in the future.
Other Resources
Australian Patient Travel Subsidy Scheme
Booklets & Guides
Basic Testing sheet for GastroEnteroPancreatic (GEP) Neuroendocrine Tumors (NETs)
Getting Diagnosed
