Adrenal Cortical Carcinoma (ACC) is a rare but aggressive type of cancer that originates in the adrenal cortex, the outer layer of the adrenal glands.
The exact cause of adrenal cortical carcinoma is unknown. However, certain genetic syndromes, such as Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome, are associated with an increased risk of developing ACC. Additionally, some genetic mutations, environmental factors, and exposure to radiation may contribute to the development of this cancer.
This cancer causes tumours that can produce excessive amounts of hormones, leading to various symptoms and complications. Tumour cells can metastasise – spread – to other parts of the body.
ACC is very rare; incidence is estimated to be around 1-2 cases per million. It can occur at any age but is most prevalent in children under five and adults aged 40-50.
Symptoms
The symptoms of Adrenal Cortical Carcinoma vary depending on the hormones produced by the tumour and whether the cancer has spread to other parts of the body. Common signs and symptoms include:
- Hormonal imbalance: Excess production of hormones, such as cortisol, aldosterone, and androgens, can lead to symptoms such as weight gain, high blood pressure, muscle weakness, and irregular menstruation. Hormonal imbalance can also cause excessive hair growth in women (hirsutism) due to increased production of androgens; in men, hormone changes may cause enlarged breast tissue and shrinking testicles.
- Abdominal pain: Discomfort or pain in the abdomen, often due to the enlargement of the tumour or pressure on surrounding organs.
- Mass effect symptoms: As the tumour grows, it can compress nearby structures, causing symptoms such as nausea, vomiting, abdominal fullness, and urinary symptoms.
- Unexplained weight loss: In some cases, individuals may experience unexplained weight loss despite increased appetite.
- Fatigue: Persistent fatigue and weakness may occur due to the metabolic demands of the cancer and hormonal imbalances.
- Fever: Some individuals may develop a fever, often as a result of inflammation or infection associated with the tumour.
- Skin changes: Pink or purple stretch marks may develop on the skin.
Diagnosis
Diagnosing adrenal cortical carcinoma involves a combination of medical history, physical examination, imaging tests, and laboratory studies. Diagnostic procedures may include imaging tests, using CT, PET, and MRI scans to visualise the size, location and extent of the tumour, tissue sample biopsies to confirm diagnosis and assess the tumour’s characteristics, and blood and urine tests to determine if the tumour is producing excess hormones.
Treatment
Treatment requires a multidisciplinary approach, which may include surgery, chemotherapy, radiation therapy and targeted therapy.
The primary treatment for localised ACC is a surgical removal of the tumour. In some cases, a complete resection of the tumour may offer a chance of cure. Chemotherapy and/or radiation therapy can be used before or after surgery to shrink the tumour, destroy cancer cells or prevent recurrence. For unresectable tumours, adrenolytic treatment with antihormonal drugs may be a treatment option.
Finally, targeted therapy for genetic mutations or molecular pathways involved in the growth and spread of ACC is an option for some individuals.
Summary
Adrenal Cortical Carcinoma is a rare but aggressive cancer that originates in the adrenal glands. Early detection, accurate diagnosis, and prompt treatment are essential for improving outcomes and quality of life for individuals affected by this condition. Continued research and advancements in treatment modalities hold promise for better outcomes and management of ACC in the future.
