Last Updated on November 26, 2025 by Bilal Hasdemir
At Liv Hospital, we know how vital it is to give top-notch care to patients from around the world. Adrenal cortical carcinoma (ACC) is a rare and aggressive cancer that starts in the adrenal gland. It affects about 1 in a million people every year.
This rare cancer grows in the adrenal cortex, the outer part of the adrenal gland. Knowing its causes, symptoms, and treatment options is key for early detection and effective care. We’re dedicated to delivering world-class healthcare with full support for our patients.
Key Takeaways
- Adrenal cortical carcinoma is a rare and aggressive cancer affecting the adrenal gland.
- It originates in the adrenal cortex, the outer layer of the adrenal gland.
- Understanding its causes, symptoms, and treatment options is vital for early diagnosis.
- Liv Hospital offers complete care and advanced medical treatments for international patients.
- Early detection and effective treatment are essential for managing adrenal cortical carcinoma.
What Is Adrenal Cortical Carcinoma?
The adrenal glands are key for making hormones. They can get cancer, called Adrenal Cortical Carcinoma. It’s important to know what this cancer is and how the adrenal glands help us stay healthy.
Definition and Prevalence
Adrenal Cortical Carcinoma is a rare tumor of the adrenal gland. It starts in the cortex, the outer layer. It’s a rare cancer, happening in about 1-2 cases per million people each year. It can affect both kids and adults, but more in some age groups.
Some people, like those with Li-Fraumeni syndrome, are more likely to get this adrenal tumor.
Anatomy of the Adrenal Glands
The adrenal glands sit on top of each kidney. They’re small and triangular. They make hormones that help with things like metabolism, blood pressure, and stress response. The glands have two parts: the cortex and the medulla.
- The cortex makes hormones like cortisol and aldosterone. These help keep blood pressure right and handle stress.
- The medulla makes adrenaline (epinephrine). This hormone helps us react quickly to danger.
Types of Adrenal Gland Cancer
It’s important to know the different types of adrenal gland cancer. This helps doctors choose the best treatment and improve patient care. Adrenal cortical carcinoma (ACC) is a rare and complex cancer. It can be classified based on its function and stage at diagnosis.
Functioning vs. Non-functioning Tumors
Adrenal gland tumors are divided into two main types: functioning and non-functioning. Functioning tumors make too much hormone, causing symptoms like Cushing’s syndrome or virilization. They produce hormones like cortisol or androgens.
Non-functioning tumors don’t make hormones. But, they can cause symptoms like pain or discomfort because of their size and location. They are often found by accident during imaging for other reasons.
Knowing if a tumor is functioning or non-functioning is key. It affects how it’s treated and managed. Functioning tumors need extra care to balance hormone levels. Non-functioning tumors might be watched or treated based on their size and risk of becoming cancerous.
Staging and Classification
Staging and classifying adrenal cortical carcinoma is vital. It helps predict outcomes and plan treatments. The ENSAT staging system is commonly used. It looks at tumor size, invasion, and metastases.
- Stage I: Tumor is less than 5 cm and completely resected.
- Stage II: Tumor is more than 5 cm and completely resected.
- Stage III: Tumor has invaded surrounding tissues or lymph nodes.
- Stage IV: Tumor has metastasized to distant organs.
Accurate staging is key for planning treatment. The classification also looks at tumor features like the Weiss score. This score helps tell if a tumor is benign or malignant.
In summary, knowing the types of adrenal gland cancer is essential for good care. By accurately diagnosing and classifying tumors, doctors can create personalized treatment plans. This meets the unique needs of each patient.
Causes and Risk Factors
Understanding the causes and risk factors of adrenal cortical carcinoma is key for early detection and treatment. Research shows that genetic mutations play a big role in this rare cancer.
Genetic Mutations
Specific genetic mutations increase the risk of adrenal cortical carcinoma. For example, mutations in the TP53 gene, a tumor suppressor, raise the risk. The TP53 mutation is linked to Li-Fraumeni syndrome, a condition that increases the risk of many cancers, including this one.
Another mutation involved is the IGF2 gene overexpression. The IGF2 gene helps cells grow and divide. Its overexpression can cause tumors to form.
Hereditary Syndromes
Certain hereditary syndromes raise the risk of adrenal cortical carcinoma. These include:
- Li-Fraumeni syndrome, caused by TP53 mutations.
- Beckwith-Wiedemann syndrome, linked to IGF2 overexpression.
People with a family history of these syndromes should know their risk. They should talk to their healthcare provider about screening options.
Other Risk Factors
Genetic mutations and hereditary syndromes are major risk factors. But other factors like environment and hormones might also play a part. The exact impact of these factors is being studied.
It’s vital for those at risk to get regular check-ups. They should also tell their healthcare provider about any symptoms. Early detection is key to managing adrenal cortical carcinoma well.
Signs and Symptoms of Adrenal Cortical Carcinoma
Adrenal cortical carcinoma shows a variety of symptoms. These can be divided into hormone-related and non-hormonal signs. Knowing these symptoms is key for early detection and treatment.
Hormone-Related Symptoms
Hormone-related symptoms happen when tumors make too many hormones. The adrenal cortex makes important hormones like cortisol, aldosterone, and androgens. Too much of these hormones can cause different health problems.
- Cushing’s Syndrome: Too much cortisol can lead to Cushing’s syndrome. This includes weight gain, high blood pressure, and skin and hair changes.
- Virilization: Too much androgen can make females grow hair, deepen their voice, and have irregular periods.
- Hyperaldosteronism (Conn’s Syndrome): Too much aldosterone can cause high blood pressure, low potassium, and muscle weakness.
Non-Hormonal Symptoms
Non-hormonal symptoms come from the tumor itself. They include:
- Abdominal pain or discomfort from the tumor growing.
- Weight changes, depending on how the tumor affects metabolism and appetite.
- Fatigue and feeling unwell.
- A noticeable lump in the abdomen in some cases.
These symptoms can differ based on the tumor’s size, location, and the patient’s health. It’s vital for those with these symptoms to see a doctor for the right diagnosis and treatment.
Hormone-Related Syndromes
Adrenal cortical carcinoma can lead to several hormone-related syndromes. This happens because the tumor can make and release different hormones or hormone precursors. These hormones can cause various symptoms.
Cushing’s Syndrome
Cushing’s syndrome is a common issue linked to this cancer. It’s caused by too much cortisol, a hormone from the adrenal cortex. Symptoms include weight gain, high blood pressure, and changes in skin and hair.
Key Features of Cushing’s Syndrome:
- Weight gain and fatty deposits
- High blood pressure
- Skin changes, including thinning and bruising
- Hormonal imbalances
Virilization and Feminization
Adrenal cortical carcinoma can also cause virilization or feminization. Virilization in females leads to male traits like hair growth and a deeper voice. Feminization in males causes female traits like breast growth and hair loss.
| Condition | Symptoms |
|---|---|
| Virilization in Females | Excessive hair growth, deepening voice, clitoral enlargement |
| Feminization in Males | Breast enlargement, loss of body hair |
Conn’s Syndrome (Hyperaldosteronism)
Conn’s syndrome, or primary aldosteronism, is caused by too much aldosterone. This hormone affects sodium and potassium levels. Symptoms include high blood pressure, low potassium, and muscle weakness. It’s less common in this cancer but affects treatment.
Managing hormone-related syndromes is key in treating adrenal cortical carcinoma. Knowing about these syndromes helps in diagnosing and treating the condition better.
Diagnosis and Testing
Diagnosing adrenal cortical carcinoma is a detailed process. It involves several tests and exams. We know it can seem overwhelming, but it’s key to finding the right treatment.
Physical Examination
The first step is a physical examination. Our doctors look for signs like abdominal swelling or weight changes. They also check your overall health for other possible problems.
Laboratory Tests
Laboratory tests are vital for diagnosing this condition. We test hormone levels, including:
- Cortisol
- Aldosterone
- Androgens
- Estrogens
High levels of these hormones can mean a tumor is present. We also check blood chemistry to see if there are any other issues related to the tumor.
Imaging Studies
Imaging studies help us see the adrenal glands and find tumors. We use:
- Computed Tomography (CT) scans: Give detailed images of the glands and nearby areas.
- Magnetic Resonance Imaging (MRI): Provides clear images to tell if a tumor is cancerous.
- Positron Emission Tomography (PET) scans: Show how active the tumor is and if it has spread.
Biopsy and Pathology
A biopsy takes a tissue sample from the tumor. Our pathologists then check it for cancer cells. They also find out the cancer type and grade. This info helps us plan the best treatment.
We know the diagnostic process can be tough and emotional. Our team offers caring support every step of the way.
Surgical Treatment Options
When it comes to treating adrenal cortical carcinoma, doctors have two main choices. They can choose between open and laparoscopic adrenalectomy. Each method has its own benefits and things to consider. The decision depends on the tumor’s size, location, and the patient’s health.
Open Adrenalectomy
Open adrenalectomy is a traditional method. It involves a bigger cut to reach the adrenal gland. This approach is good for removing big tumors and the tissue around them. It’s key when the tumor is large or might spread.
Advantages of Open Adrenalectomy:
- Allows for en bloc resection of large tumors
- Better visualization and access to surrounding structures
- Can be used for tumors that are not suitable for laparoscopic surgery
Laparoscopic Approaches
Laparoscopic adrenalectomy is a newer, less invasive method. It uses small cuts for instruments and a camera. This method leads to less pain, shorter hospital stays, and quicker healing.
Benefits of Laparoscopic Adrenalectomy:
- Reduced postoperative pain and discomfort
- Shorter hospital stays and faster recovery
- Less visible scarring
But, laparoscopic surgery might not work for everyone. It’s not best for big or invasive tumors. Doctors must decide based on each patient’s needs and the tumor’s specifics.
| Surgical Approach | Advantages | Disadvantages |
|---|---|---|
| Open Adrenalectomy | Better for large or invasive tumors, allows for en bloc resection | Larger incision, more postoperative pain, longer recovery |
| Laparoscopic Adrenalectomy | Less postoperative pain, shorter hospital stay, less scarring | May not be suitable for large or invasive tumors, requires specialized skills |
In conclusion, choosing between open and laparoscopic surgery for adrenal cortical carcinoma depends on many factors. These include the tumor’s size and location, and the patient’s health. A team of doctors works together to find the best surgery for each patient.
Medical Therapies
Medical therapies offer many options for those with adrenal cortical carcinoma. These treatments help manage the disease, slow tumor growth, and ease symptoms.
Chemotherapy Protocols
Chemotherapy uses drugs to kill cancer cells. For adrenal cortical carcinoma, a mix of drugs is often used. Etoposide, doxorubicin, and cisplatin are common choices. The right treatment depends on the disease’s stage and the patient’s health.
Clinical trials help find the best chemotherapy for this cancer. Patients should talk to their doctors about joining trials.
Mitotane Therapy
Mitotane is a special drug for treating this cancer. It lowers steroid production in the adrenal gland, slowing tumor growth. It’s often paired with surgery or chemotherapy.
It’s important to check mitotane levels to make sure it’s working right and to avoid side effects.
Hormone Management
Hormone management is key for treating this cancer, mainly for tumors that make too many hormones. Cushing’s syndrome, caused by too much cortisol, is a common issue. Medications help control hormone levels.
- Medications to control cortisol production
- Treatments to address other hormonal imbalances
- Regular monitoring of hormone levels
Good hormone management can greatly improve life for those with this cancer.
Multidisciplinary Management Approach
Treating adrenal cortical carcinoma needs a team effort. It’s a complex disease that requires many medical experts. They work together to give the best care.
The Treatment Team
The team for treating this disease includes surgeons, oncologists, and endocrinologists. Surgeons remove tumors. Oncologists handle chemotherapy and other treatments. Endocrinologists manage hormone issues.
It’s key for these experts to work together. They share information to make a good treatment plan. This ensures all parts of a patient’s care are covered.
Coordinating Care
Coordinating care is very important. We listen to each patient’s needs and create a plan just for them. This plan is shared with the whole team.
Good coordination means everyone works together. This includes doctors, nurses, social workers, and more. They all help make sure patients get the care they need.
| Specialist | Role in Treatment |
|---|---|
| Surgeon | Surgical removal of tumors |
| Oncologist | Chemotherapy and medical therapies |
| Endocrinologist | Managing hormone-related issues |
By working together, we aim for the best results for our patients with adrenal cortical carcinoma.
Prognosis and Follow-up Care
The outlook for adrenal cortical carcinoma changes a lot based on several things. These include the tumor’s stage and type. Knowing the prognosis helps both patients and doctors make better treatment and follow-up plans.
Survival Rates and Recurrence
Survival chances for adrenal cortical carcinoma depend on when it’s found. People diagnosed early tend to do better than those found later. The 5-year survival rate is higher for those with localized disease than for those with spread.
Key factors influencing survival rates include:
- Tumor size and stage at diagnosis
- Presence of metastasis
- Effectiveness of initial treatment
- Histological characteristics of the tumor
Recurrence is a big worry for those with adrenal cortical carcinoma, even after treatment. Regular check-ups are key to catching recurrence early.
Long-term Monitoring
Keeping an eye on adrenal cortical carcinoma over time is vital. This means regular scans, blood tests, and doctor visits to watch for signs of trouble.
Effective long-term monitoring involves:
- Regular CT or MRI scans to monitor for recurrence or metastasis
- Laboratory tests to assess hormone levels and detect any abnormalities
- Clinical evaluations to monitor for symptoms or signs of recurrence
We stress the need for a team approach to follow-up care. This team should include endocrinologists, oncologists, surgeons, and others as needed.
Understanding the prognosis and sticking to a detailed follow-up plan can help patients with adrenal cortical carcinoma get the best results.
Conclusion
Understanding adrenal cortical carcinoma is key for early detection and effective treatment. We’ve looked into its causes, symptoms, diagnosis, and treatment options. This rare and aggressive cancer needs accurate diagnosis and ongoing research.
Effective treatment plans are vital, including surgery and medical therapies. A team of specialists is important for managing the cancer. They work together to support patients throughout their treatment.
As research improves, we’re dedicated to top-notch healthcare for international patients. By keeping up with the latest medical knowledge and technology, we aim to better patient outcomes. This will also improve their quality of life.
FAQ
What is adrenal cortical carcinoma?
Adrenal cortical carcinoma (ACC) is a rare and aggressive cancer. It affects the adrenal gland. This gland is vital for producing hormones that control our body’s functions.
What are the symptoms of adrenal cortical carcinoma?
Symptoms of ACC vary. They depend on the tumor’s type and size. Hormone-related symptoms include Cushing’s syndrome and virilization. Non-hormonal symptoms are abdominal pain and weight gain.
How is adrenal cortical carcinoma diagnosed?
Diagnosing ACC involves several steps. It starts with a physical exam and hormone level tests. Imaging studies like CT and MRI scans are used to see the tumor. A biopsy and pathology confirm the diagnosis.
What are the treatment options for adrenal cortical carcinoma?
Treatment for ACC includes surgery to remove the tumor. Chemotherapy and mitotane therapy are also used. Hormone management helps with hormonal imbalances caused by the tumor.
What is the prognosis for adrenal cortical carcinoma?
ACC’s prognosis varies. It depends on the tumor’s stage and type. Survival times range from a few months to several years. Long-term monitoring is key to detect recurrence and manage long-term effects.
What is the role of genetic mutations in adrenal cortical carcinoma?
Genetic mutations, like TP53 and IGF2, play a role in ACC. Hereditary syndromes, such as Li-Fraumeni syndrome, also increase the risk of developing the disease.
How is adrenal cortical carcinoma staged and classified?
ACC is staged and classified based on the tumor’s size and extent. The presence of metastasis is also considered. The ENSAT staging system is used.
What is the importance of a multidisciplinary management approach for adrenal cortical carcinoma?
A team of specialists, including surgeons and oncologists, is essential. They work together to provide complete care. This ensures the best outcomes for patients with ACC.
Can adrenal cortical carcinoma be treated with surgery alone?
Surgery is often the first treatment for ACC. But, chemotherapy and mitotane therapy may be needed. They help control the tumor’s growth and manage symptoms.
How can hormone-related syndromes associated with adrenal cortical carcinoma be managed?
Hormone-related syndromes, like Cushing’s syndrome, can be managed. Hormone management therapies aim to balance hormone levels caused by the tumor.
References
National Cancer Institute — Adrenocortical Carcinoma Treatment (PDQ®) — Patient Version
https://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq
Osmosis — Adrenal cortical carcinoma
