Adrenocortical Carcinoma

Adrenocortical carcinoma, also known as ACC, is a rare and aggressive form of cancer that affects the adrenal glands. These glands are located on top of the kidneys and are responsible for producing hormones that regulate various bodily functions. Adrenocortical carcinoma occurs when abnormal cells develop in the outer layer of the adrenal glands, known as the adrenal cortex. This type of cancer can be challenging to diagnose as its symptoms can be vague and non-specific.

If you suspect you or someone else is experiencing Adrenocortical carcinoma, it is crucial to seek immediate medical attention by calling emergency services or consult with a Cancer Specialist.

Causes of Adrenocortical Carcinoma

• Genetic Factors:

  • Inherited Syndromes: Some genetic disorders are associated with a higher risk of ACC. These include:
    • Li-Fraumeni Syndrome: A hereditary condition that increases the risk of developing several types of cancer, including ACC.
    • Beckwith-Wiedemann Syndrome: A growth disorder that can lead to an increased risk of various tumors, including ACC.
    • Familial Adenomatous Polyposis (FAP): An inherited condition that predisposes individuals to developing colorectal cancer and may also increase the risk of ACC.
    • Multiple Endocrine Neoplasia Type 1 (MEN1): A genetic disorder that can lead to tumors in endocrine glands, including the adrenal glands.

• Hormonal Imbalance:

  • Overproduction of adrenal hormones, such as cortisol, aldosterone, and sex hormones, can sometimes lead to ACC. Hormonal imbalances can result from the abnormal functioning of the adrenal glands, potentially leading to cancer development.

• Previous History of Adrenal Tumors:

  • People who have had benign adrenal tumors or other types of adrenal disorders may be at a slightly higher risk for developing ACC.

• Age and Gender:

  • ACC can occur at any age, but it is more common in adults between the ages of 40 and 50. It is also slightly more common in women than men.

• Environmental Factors:

  • While the direct environmental causes of ACC are not clear, exposure to certain carcinogens or chemicals may increase the risk of developing various types of cancers, including those in the adrenal glands.

• Family History:

  • A family history of ACC or other types of cancer may also increase the risk, suggesting a genetic predisposition to developing the disease.

Risk Factors of Adrenocortical Carcinoma

• Genetic mutations (e.g., Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome)
• Age (typically 40-60 years)
• Gender (slightly more common in females)
• Hormonal imbalances (e.g., Cushing's syndrome, hyperaldosteronism)
• Family history of cancer
• Obesity
• Previous cancer treatments (e.g., radiation to abdomen or pelvis)

Symptoms of Adrenocortical Carcinoma

Abdominal pain or mass: A lump or discomfort in the abdomen due to the growing tumor.

Unexplained weight loss: Significant and unexplained weight loss.

Excessive hair growth (hirsutism): Increased hair growth, particularly in women, due to an overproduction of androgens.

Changes in menstrual cycle: Irregular or absent periods in women, resulting from hormonal imbalances.

High blood pressure (hypertension): Caused by excessive production of aldosterone or cortisol.

Excessive thirst and urination: Due to high levels of aldosterone.

Fatigue: Persistent tiredness or weakness, common with any form of cancer.

Cushing's syndrome symptoms:

• Round, red face (moon face)
• Thinning skin that bruises easily
• Stretch marks on the skin (striae)
• Muscle weakness

Virility in women: Voice deepening, increased muscle mass, and male-pattern baldness due to excess androgen production.

Hyperglycemia: Elevated blood sugar levels from excess cortisol production.

Chest pain or shortness of breath: If the cancer has spread to the lungs or other organs.

Loss of appetite: Leading to unintended weight loss.

Diagnosis for Adrenocortical Carcinoma

• Medical History and Physical Exam:

  • A healthcare provider will evaluate symptoms, family history, and perform a physical examination, including checking for signs of hormone imbalances.

• Blood Tests:

  • Hormonal Testing: To measure levels of adrenal hormones (cortisol, aldosterone, and androgens) to detect any hormone overproduction.
  • Corticosteroid and ACTH levels: Elevated cortisol levels and suppressed ACTH may indicate Cushing’s syndrome associated with ACC.

• Imaging Studies:

  • CT Scan (Computed Tomography): A contrast-enhanced CT scan of the abdomen is commonly used to identify and evaluate the size, shape, and spread of the tumor in the adrenal glands.
  • MRI (Magnetic Resonance Imaging): Often used to further evaluate the adrenal tumor and its relationship with surrounding structures, especially if the CT scan results are unclear.
  • PET Scan (Positron Emission Tomography): Can help assess whether the cancer has spread (metastasized) to other parts of the body.

• Ultrasound:

  • Used in some cases to detect the presence of an adrenal mass, though it is less detailed than CT or MRI.

• Biopsy:

  • Needle Biopsy: A small sample of the tumor is taken to examine under a microscope for confirmation of cancerous cells.
  • Biopsy is typically avoided if the tumor is large or there is concern about spreading cancer cells, and imaging results are sufficient for diagnosis.

• Genetic Testing:

  • To identify underlying genetic syndromes (e.g., Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome) that may predispose individuals to ACC.

• Histopathological Examination:

  • A biopsy or surgical resection sample is examined under a microscope to confirm ACC, often revealing specific patterns of cells associated with adrenocortical carcinoma.

• Adrenal Vein Sampling:

  • In some cases, this test is used to differentiate between primary adrenal disease and other conditions if there is a suspicion of metastatic disease from other cancers.

Treatments for Adrenocortical Carcinoma

• Surgery:

  • Adrenalectomy: The primary treatment for localized ACC is the surgical removal of the affected adrenal gland (or both, if necessary). In some cases, nearby lymph nodes or tissues may also be removed.
  • Laparoscopic Adrenalectomy: Minimally invasive surgery may be used in certain cases where the tumor is smaller or confined.

• Radiation Therapy:

  • Used to shrink the tumor before surgery or to treat areas where the cancer has spread (metastases). It may be used as palliative care for patients with inoperable tumors or recurrent ACC.

• Chemotherapy:

  • Mitotane: A drug that specifically targets adrenal cancer cells and is often used in combination with other chemotherapy agents to treat ACC. It's the main chemotherapy treatment.
  • Other Chemotherapy Drugs: Drugs like etoposide, doxorubicin, or cisplatin may be used, particularly if the cancer has spread to other parts of the body.

• Hormonal Therapy:

  • Steroid Replacement Therapy: Patients who undergo surgery to remove the adrenal glands may require steroid medications to replace hormones normally produced by the adrenal glands, such as cortisol.
  • Inhibitors of Hormone Production: If ACC produces excess hormones (like cortisol or aldosterone), medications like mifepristone (to block cortisol) or spironolactone (to block aldosterone) may be used to control symptoms.

• Targeted Therapy:

  • Checkpoint Inhibitors: Immunotherapy treatments that aim to boost the body’s immune response against cancer cells.
  • TKI (Tyrosine Kinase Inhibitors): Drugs like sunitinib may be considered in clinical trials for advanced ACC to target cancer growth at the molecular level.

• Palliative Care:

  • For patients with advanced ACC or metastasis, palliative treatments may be offered to manage symptoms, improve quality of life, and ease pain.
  • This may include pain management, radiation therapy for metastasis, and emotional support.

• Liver Transplantation:

  • In very rare cases where the tumor has spread to the liver but remains localized to that organ, a liver transplant may be considered as part of a treatment strategy.

Preventive Measures for Adrenocortical Carcinoma

• Genetic Counseling and Testing:

  • For individuals with a family history of genetic syndromes (e.g., Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, or Familial Adenomatous Polyposis), genetic counseling and testing can help identify inherited risks for ACC and guide preventive care.

• Regular Screening for At-Risk Populations:

  • People with inherited genetic conditions that increase the risk of ACC may benefit from early screening, including imaging tests (CT scans or MRIs) and hormonal level checks, to detect tumors at an early, more treatable stage.

• Hormonal Regulation:

  • Monitoring and managing hormone imbalances, such as those related to conditions like Cushing’s syndrome or hyperaldosteronism, may help prevent the development of tumors in the adrenal glands, though this is not a guarantee.

• Healthy Lifestyle:

  • Balanced Diet: A healthy diet rich in fruits, vegetables, and whole grains may help support overall health and reduce the risk of certain cancers.
  • Regular Physical Activity: Staying physically active helps maintain a healthy weight, reduce inflammation, and support the body’s immune system, potentially lowering cancer risk.
  • Avoiding Exposure to Carcinogens: Limiting exposure to environmental toxins, chemicals, and carcinogens that could potentially damage the adrenal glands or increase the risk of cancer.

• Early Detection and Regular Health Check-ups:

  • Routine medical check-ups, especially for those with a family history of endocrine cancers or genetic predisposition, can help catch early signs of adrenal issues or tumors.
  • Monitoring for abnormal symptoms such as unexplained weight loss, hormonal imbalances, or abdominal pain can prompt early evaluation and diagnosis.

• Avoiding Excessive Steroid Use:

  • Long-term use of corticosteroids or other medications that affect adrenal gland function should be monitored carefully, as these drugs may have side effects that impact adrenal health. Always follow medical advice when using steroids.

Do's & Don’t's

When it comes to dealing with adrenocortical carcinoma, there are certain do's and don'ts that can help patients and their loved ones navigate this challenging journey. By following these guidelines, individuals can better manage their condition and improve their overall well-being. 

If you suspect you or someone else is experiencing Adrenocortical carcinoma, it is crucial to seek immediate medical attention by calling emergency services or consult with a Cancer Specialist.