About Adrenocortical Carcinoma (Adrenal Cancer)
Adrenocortical carcinoma is a rare cancer that affects the outside of the adrenal glands, which are located on top of each kidney. Adrenocortical carcinoma is caused by the uncontrolled growth and division of cells in the adrenal cortex, which is the outer layer of the adrenal glands. This abnormal cell growth can lead to the formation of tumors.
The main risk factor is genetic predispositions. Some genetic changes can increase the risk of developing adrenocortical carcinoma. Genetic mutations may be inherited, others are changes that occur but are not inherited.
- For example, changes in genes such as TP53 and IGF2 have been associated with this cancer.
- Having a positive family history of adrenocortical carcinoma or other related cancers can also increase the risk.
- Inherited conditions with an increased risk of developing adrenocortical carcinoma include:
- Familial adenomatous polyposis (FAF)
- Li-Fraumeni syndrome
- Lynch syndrome
- Multiple endocrine neoplasia (MEN1)
- Von Hippel-Lindau (HVL) syndrome
Modifiable risk factors for adrenocortical carcinoma have not been defined. Some researchers believe smoking may be a risk factor.
People with adrenocortical carcinoma may not have symptoms and the tumor is discovered during medical care for other conditions.
When symptoms occure, they may include:
- Abdominal pain: Some people with ACC may experience pain in their abdomen. This pain can be persistent and may worsen over time.
- Hypertension (high blood pressure): ACC can cause an increase in blood pressure, leading to hypertension. This symptom may be present in some individuals with ACC.
- Weight gain: Another common symptom of ACC is unexplained weight gain. This weight gain may occur despite maintaining a healthy diet and exercise routine.
- Frequent urination: ACC can affect the normal functioning of the adrenal glands, leading to increased urine production and frequent urination.
- Deepening of the voice: In some cases, ACC can cause a deepening of the voice, particularly in females. This is due to the excess secretion of hormones from the adrenal glands.
Additional symptoms may include:
- Muscle weakness: ACC can cause muscle weakness, making it difficult to perform everyday activities.
- Osteoporosis: The overproduction of cortisol by ACC can lead to weakened bones and an increased risk of osteoporosis.
- Fatigue: As ACC progresses, individuals may experience extreme tiredness or fatigue, which can impact their daily life and activities.
It's important to note that early diagnosis and treatment of ACC are crucial for a positive outlook and improved quality of life. Delaying treatment can make managing the cancer more challenging. Additionally, if ACC spreads to other parts of the body, the 5-year survival rate drops to 36-46%.
To diagnose adrenocortical carcinoma, healthcare providers may perform a series of examinations, tests, and procedures. These include:
- Physical Exam: During a physical exam, the healthcare provider will look for physical signs of a medical problem, such as pain, swelling, or the presence of masses within the body.
- Lab Tests: Lab tests may be ordered to check a sample of blood, urine, or body tissues. These tests help diagnose the disease and determine its severity. For ACC, lab tests may include checking hormone levels in the blood and urine.
- Imaging Tests: Imaging tests allow doctors to look inside the body for clues to help diagnose and determine the stage or severity of ACC. Common imaging tests used for ACC include:
- Ultrasound: Uses sound waves to create images of the adrenal glands.
- CT Scan: Provides detailed cross-sectional images of the body.
- X-ray: Uses radiation to create images of the inside of the body.
- PET Scan: Uses a radioactive substance to highlight areas of abnormal cell activity.
- MRI Scan: Uses powerful magnets and radio waves to create detailed images.
- Biopsy: A biopsy involves taking a small sample from the tumor with a needle. This sample is then examined under a microscope by a pathologist to determine if it is ACC or another type of tumor.
In addition to these examinations, tests, and procedures used for diagnosis, there are additional examinations, tests, and procedures that can help determine the stage or severity of ACC. These may include:
- Genetic Testing: Since ACC is considered a genetic disease, healthcare providers may recommend genetic testing to identify any changes in genes or chromosomes that may be associated with ACC.
- Clinical Procedures: Depending on the specific symptoms and body systems being evaluated, healthcare providers may recommend additional clinical procedures. These procedures can vary widely and may be performed by nurses, physician's assistants, or lab technicians before results are reviewed by your doctor.
Remember that it's important to follow up with your healthcare provider if any symptoms worsen or change after your physical exam. They will guide you through any necessary examinations, tests, and procedures needed for an accurate diagnosis and staging of adrenocortical carcinoma.
The goals of treatment for adrenocortical carcinoma (ACC) are to remove as much of the tumor as possible, cure the cancer, and prevent recurrence. To achieve these goals, several types of treatments and therapies are recommended:
- Surgery: Surgery is the primary treatment for ACC. The goal of surgery is to remove as much of the tumor as possible. In some cases, small ACCs can be completely cured with surgery alone. However, for larger tumors or when the cancer has spread to other parts of the body, surgery may be combined with other treatments.
- Chemotherapy: Chemotherapy is used in combination with surgery when the ACC tumors are large or when the cancer cells have spread to other parts of the body. Chemotherapy involves the use of drugs that kill cancer cells or prevent them from growing. It can help shrink tumors and reduce the risk of recurrence.
- Radiation Therapy: Radiation therapy is not commonly used for adrenocortical tumors because they are generally considered to be resistant to radiation. However, in some cases, radiation therapy may be used as part of a treatment plan, especially in pediatric patients with adrenocortical tumors.
- Adjuvant Therapy: Adjuvant therapy refers to additional treatments given after surgery to reduce the risk of recurrence. In ACC, adjuvant therapy often involves the use of a medication called mitotane. Mitotane is a type of chemotherapy drug that can help prevent the regrowth of cancer cells and reduce the risk of recurrence.
- Health Behavior Changes: Making certain lifestyle changes can also play a role in ACC treatment. These changes may include maintaining a healthy weight, eating a balanced diet, exercising regularly, and avoiding tobacco and excessive alcohol consumption. These health behavior changes can help support overall health and well-being during and after treatment.
It's important for individuals diagnosed with ACC to work closely with their healthcare team to determine the most appropriate treatment plan based on their specific situation and needs.
The natural progression of ACC can vary depending on various factors, including the stage of the cancer and individual characteristics. Early diagnosis and treatment are crucial in improving the prognosis and quality of life for individuals with ACC.
Complications commonly associated with ACC are often related to the overproduction of cortisol, a hormone produced by the adrenal glands. These complications may include:
- Muscle weakness: Excess cortisol can lead to muscle weakness, making it difficult for individuals to perform daily activities.
- Osteoporosis: Prolonged exposure to high levels of cortisol can weaken bones, leading to an increased risk of fractures.
- Fatigue: ACC can cause chronic fatigue, making individuals feel tired and lacking energy.
Treatment for ACC aims to remove as much of the cancer as possible and manage its progression. The impact of treatment for ACC varies depending on factors such as the stage of the cancer and individual response to treatment. Early diagnosis and prompt treatment increase the chances of curing ACC and improving overall outcomes. Delaying treatment can make managing the cancer more challenging.
It's important to note that each case of ACC is unique, and treatment plans should be tailored to individual needs and circumstances. If you or someone you know has been diagnosed with ACC or is experiencing symptoms related to adrenal gland issues, it's essential to consult a healthcare professional for an accurate diagnosis and appropriate treatment options. They can provide personalized guidance based on your specific situation.