Adrenocortical carcinoma
At a Glance
Adrenocortical carcinoma is a rare and aggressive cancer that forms in the outer layer of the adrenal glands and often produces excess hormones that affect the entire body.
This rare condition affects only about one in a million people annually and typically follows a bimodal age distribution, occurring most often in children under five and adults between their 40s and 50s.
It is a serious, chronic condition that requires immediate and often long-term treatment to manage tumor growth and control hormonal imbalances.
The outlook varies significantly by stage, with early detection and complete surgical removal offering the best chance for long-term survival, while advanced disease generally carries a poor prognosis.
How It Affects You
Adrenocortical carcinoma is a rare malignancy that originates in the outer layer of the adrenal glands, which are located on top of the kidneys in the abdomen. As the tumor grows, it can compress nearby organs, causing pain and fullness, while functioning tumors release excessive hormones that trigger systemic changes throughout the body. Advanced stages may spread to the lungs, liver, or bones.
- Weight gain, facial roundness, and skin changes due to excess cortisol
- Abdominal swelling, pain, or a palpable lump
- Unexpected hair growth or voice deepening from androgen excess
Causes and Risk Factors
Underlying Causes
The exact cause of adrenocortical carcinoma is often unknown, but it begins when DNA mutations occur in the cells of the adrenal cortex. These mutations cause the cells to multiply uncontrollably and form a tumor. While most cases are sporadic, meaning they happen by chance, a significant number are linked to inherited genetic changes that affect how cells grow and divide.
Genetic Risk Factors
A major risk factor for developing this cancer is the presence of certain hereditary syndromes. People with Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Lynch syndrome, or Multiple Endocrine Neoplasia type 1 (MEN1) have a higher likelihood of developing adrenal tumors. In children, this cancer is frequently associated with these underlying genetic conditions.
Prevention Strategies
Because most cases of adrenocortical carcinoma are sporadic and the exact environmental triggers are not well defined, there is no known way to prevent the disease in the general population. For individuals with a known family history of adrenal cancer or associated genetic syndromes, genetic counseling and regular surveillance screenings are recommended. These screenings do not prevent the cancer but can help detect it at an earlier, more treatable stage.
Diagnosis, Signs, and Symptoms
Signs and Symptoms
Symptoms vary depending on whether the tumor is "functional" (producing hormones) or "non-functional." Functional tumors, which are more common, can cause specific syndromes based on the hormone released. Excess cortisol leads to Cushing syndrome, characterized by weight gain in the face and abdomen, purple stretch marks, and muscle weakness. Excess androgens can cause virilization, leading to facial hair growth and a deep voice in women or early puberty in children. Excess estrogen may cause breast enlargement in men. Non-functional tumors do not produce hormones and are often detected only when they grow large enough to cause abdominal pain, a feeling of fullness, or a palpable lump.
Diagnostic Tests
Clinicians use a combination of physical exams, imaging, and laboratory tests to diagnose the condition. Blood and urine tests are essential to measure levels of adrenal hormones like cortisol, aldosterone, and androgens. Imaging scans, such as CT (Computed Tomography) or MRI (Magnetic Resonance Imaging), help determine the size and location of the tumor and whether it has spread. A PET scan may be used to evaluate metabolic activity. A biopsy is typically avoided if the tumor looks like adrenal cancer on imaging, as piercing the tumor can spread cancer cells; instead, diagnosis is often confirmed after surgical removal.
Differential Diagnosis
Doctors must distinguish adrenocortical carcinoma from benign adrenal adenomas, which are common non-cancerous lumps. They also rule out other malignancies such as pheochromocytoma (a tumor of the adrenal medulla) or cancers that have spread to the adrenal gland from the lungs, breast, or kidneys.
Treatment and Management
Surgical Procedures
The primary and most effective treatment is an adrenalectomy, a surgery to completely remove the affected adrenal gland. If the cancer has spread to nearby organs like the kidney or liver, parts of those organs may also need to be removed. Surgeons typically prefer an open operation rather than laparoscopic surgery for large or suspected cancerous tumors to ensure the entire mass is removed without rupturing it, which could spread cancer cells.
Medications and Chemotherapy
After surgery, or for advanced cases, a drug called mitotane is often prescribed. Mitotane specifically targets adrenal cortex cells and can block hormone production while treating remaining cancer cells. Standard chemotherapy drugs, such as cisplatin, doxorubicin, and etoposide, may also be used, particularly for metastatic disease. Patients with functional tumors also receive medications to block the effects of excess hormones and manage symptoms like high blood pressure or diabetes.
Monitoring and Follow-Up
Long-term monitoring is critical due to the risk of recurrence. Patients require regular blood tests to check hormone levels and imaging scans every few months for several years. This routine helps detect any return of the cancer early when it might still be treatable.
When to Seek Medical Care
You should see a doctor if you notice unexplained weight gain (especially in the face and trunk), sudden hair growth on the face or body, or a lump in the abdomen. Immediate medical attention is needed if you experience severe abdominal pain or signs of hormonal crisis, such as severe weakness or confusion. Routine follow-up appointments are essential for anyone with a history of adrenal nodules or genetic syndromes linked to adrenal cancer.
Severity and Prognosis
Severity and Disease Course
Adrenocortical carcinoma is a severe and life-threatening condition. It is often aggressive, with a high potential to invade nearby tissues and spread (metastasize) to the lungs, liver, and bones. The disease course varies; some tumors grow rapidly, while others progress more slowly. Functional tumors may be caught earlier due to visible hormonal symptoms, whereas non-functional tumors are often diagnosed at a later, more dangerous stage because they remain silent until they are large.
Prognosis and Survival
The prognosis depends heavily on the stage at diagnosis and whether the surgeon can completely remove the tumor. Stage 1 and 2 cancers, which are confined to the adrenal gland, have a much better outlook, with five-year survival rates ranging from 50% to over 60%. However, once the cancer spreads to lymph nodes (Stage 3) or distant organs (Stage 4), survival rates drop significantly, often falling below 15% for widespread disease. Younger age and successful initial surgery are generally associated with better outcomes.
Complications
Complications can arise from both the cancer and its hormonal effects. Excess hormones can lead to severe hypertension, diabetes, and osteoporosis (brittle bones). If the tumor spreads, it can impair the function of the lungs or liver. Long-term treatment with mitotane also carries risks, including adrenal insufficiency, where the body no longer makes enough necessary hormones, requiring lifelong replacement therapy.
Impact on Daily Life
Daily Activities and Coping
Living with adrenocortical carcinoma often involves managing the physical recovery from major surgery and the side effects of medications like mitotane, which can cause nausea, fatigue, and neurological symptoms. Patients may need to adjust their work or school schedules to accommodate fatigue and medical appointments. Nutrition plays a key role, especially for those managing diabetes or weight changes caused by hormonal imbalances.
Emotional and Social Impact
The diagnosis of a rare and aggressive cancer can be isolating and anxiety-inducing. Visible changes in appearance from excess hormones, such as weight gain or hair growth, can also affect self-esteem and social interactions. Connecting with support groups for rare cancers can provide valuable community and resources.
Questions to Ask Your Healthcare Provider
Bringing a list of questions to appointments can help clarify the path forward.
- What is the specific stage of my cancer, and has it spread?
- Is surgery an option for me, and can the entire tumor be removed?
- What are the side effects of mitotane, and how can I manage them?
- Should I undergo genetic testing for hereditary syndromes?
- How often do I need imaging scans and blood tests during follow-up?
Common Questions and Answers
Q: Is adrenocortical carcinoma curable?
A: Yes, it can be cured if it is detected early and completely removed with surgery. However, because it is aggressive and often found at later stages, the risk of recurrence is high, and long-term monitoring is always necessary.
Q: Is this cancer hereditary?
A: While most cases occur sporadically without a family link, a significant portion (especially in children) is associated with genetic conditions like Li-Fraumeni syndrome. Genetic counseling is often recommended for patients and their families.
Q: What is the difference between an adrenal adenoma and adrenocortical carcinoma?
A: An adrenal adenoma is a common, benign (non-cancerous) tumor that does not spread, whereas adrenocortical carcinoma is a malignant (cancerous) growth that invades tissue and can spread to other parts of the body.
Q: Why does this cancer cause physical changes like weight gain?
A: Many of these tumors produce excess cortisol. High levels of cortisol lead to Cushing syndrome, which changes how the body stores fat, resulting in weight gain in the face, neck, and abdomen.