Last Updated on November 26, 2025 by Bilal Hasdemir

Getting a rare cancer diagnosis can be scary and confusing. At Liv Hospital, we aim to clear up any doubts. Our team of experts is here to guide you through this tough time.

Adrenal cortical carcinoma is a rare cancer that starts in the adrenal gland. We’ll look into what it is, how common it is, and its effects. We’ll also cover how it’s diagnosed and treated.

Key Takeaways

• Adrenal cortical carcinoma is a rare and complex type of cancer.
• Accurate diagnosis is key for effective treatment.
• Liv Hospital offers top-notch care and puts patients first.
• Our team is committed to helping you through this tough time.
• We use the latest medical protocols and have international expertise.

Understanding Adrenal Cortical Carcinoma Cancer

Adrenal cortical carcinoma is a rare cancer that starts in the adrenal cortex. This is the outer layer of the adrenal glands. We will look into its definition, how common it is, and its effects on the body. We will also compare benign and malignant adrenal tumors.

Definition and Prevalence in the United States

Adrenal cortical carcinoma is a cancer that grows in the adrenal cortex. It’s quite rare, with about 1-2 cases per million people each year in the U.S. The American Cancer Society says around 300 new cases are found in the U.S. every year.

This cancer is hard to diagnose and treat because it’s so rare. It can happen at any age but is most common in kids under 5 and adults between 40 and 50.

The Role of the Adrenal Cortex in the Body

The adrenal cortex makes important hormones like cortisol and aldosterone. These hormones help with metabolism, immune response, and blood pressure. Cortisol helps us deal with stress, and aldosterone helps balance sodium and potassium.

The adrenal cortex is key for keeping our body balanced and healthy. Problems with it, like cancer, can cause big health issues.

Difference Between Benign and Malignant Adrenal Tumors

Not all adrenal tumors are cancerous; some are benign. Benign tumors, like adenomas, are usually not cancer and might not cause symptoms unless they make too much hormone. Malignant tumors, like adrenal cortical carcinoma, are cancerous and can spread to other parts of the body.

Telling benign from malignant tumors is important for treatment. Doctors use tests like imaging and biopsies to figure this out.

We will keep exploring adrenal cortical carcinoma. We will look into how it’s diagnosed and treated in the next sections.

Anatomy and Function of the Adrenal Glands

The adrenal glands sit atop each kidney. They are key organs that make many hormones. These glands help keep us healthy by controlling our body’s functions.

Structure of the Adrenal Glands

The adrenal glands have a special structure for their job. Each gland has two main parts: the outer cortex and the inner medulla. The adrenal cortex makes hormones like cortisol and aldosterone. These hormones help with metabolism, immune response, and keeping the right balance of electrolytes.

The adrenal medulla makes adrenaline (epinephrine) and noradrenaline (norepinephrine). These hormones help us deal with stress.

Normal Hormonal Functions and Regulation

The hormones from the adrenal glands are controlled by a complex system. This system involves the hypothalamus and pituitary gland. For example, the hypothalamic-pituitary-adrenal (HPA) axis regulates cortisol production. This ensures our hormones stay balanced, which is key for health.

Keeping hormones in check is a complex task. It involves many feedback loops. For instance, high cortisol levels tell the hypothalamus and pituitary gland to make less CRH and ACTH. This balance keeps our body’s functions healthy.

Types and Classification of Adrenal Cortical Carcinoma Cancer

It’s important to know the different types of adrenal cortical carcinoma for diagnosis and treatment. This disease is diverse, with tumors showing different traits and behaviors.

Functioning vs. Nonfunctioning Tumors

Adrenal cortical carcinomas are divided into functioning and nonfunctioning tumors. Functioning tumors make hormones that cause symptoms. Nonfunctioning tumors don’t make hormones or make them in small amounts.

Functioning tumors are often found early because of hormone symptoms. For example, tumors that make cortisol can cause Cushing’s syndrome. This includes weight gain, high blood pressure, and other metabolic issues.

Nonfunctioning tumors grow bigger before they’re found. They may cause symptoms like abdominal pain or discomfort.

Hormone-Secreting Variants

Adrenal cortical carcinomas that make hormones can cause different symptoms. The most common hormones are cortisol, androgens, and aldosterone.

The symptoms of hormone-secreting ACC vary. For instance, androgen-secreting tumors can make women grow hair and change their voice. Aldosterone-secreting tumors can lead to high blood pressure and low potassium levels.

Knowing which hormones a tumor makes is key to managing the disease. It helps doctors tailor treatments to fix the tumor and balance hormone levels.

Causes and Risk Factors

The causes of adrenal cortical carcinoma are complex. They involve both genetic and environmental factors. Knowing these risk factors helps us find people at higher risk. It also helps us try to prevent this aggressive cancer.

Genetic Predispositions and Hereditary Syndromes

Some genetic syndromes raise the risk of getting adrenal cortical carcinoma. For example, Li-Fraumeni syndrome is linked to a TP53 gene mutation. Other conditions like Beckwith-Wiedemann syndrome and Multiple Endocrine Neoplasia Type 1 (MEN1) also increase the risk.

TP53 Mutations and Other Genetic Factors

TP53 mutations are common in adrenal cortical carcinoma. These mutations can cause cells to grow out of control. Other genetic changes, like those in cell cycle and DNA repair genes, also play a role.

Environmental and Lifestyle Risk Factors

Genetic factors are key, but environment and lifestyle also matter. Exposure to some chemicals and radiation might increase risk. More research is needed to understand their impact.

Knowing the causes and risk factors helps us find and help those at risk. This way, we can lower the number of cases of this aggressive cancer through early detection and prevention.

Signs and Symptoms of Adrenal Cancer

It’s important to know the signs of adrenal cortical carcinoma early. This cancer can show in different ways, often because of hormone changes.

Symptoms Related to Hormone Overproduction

One key sign of this cancer is too much hormone production. This can cause many symptoms. These include:

• Cushing’s Syndrome: This is marked by weight gain, high blood pressure, and skin and hair changes.
• Virilization: Women may grow male features like too much hair and a deeper voice.
• Feminization: Men may grow female features, like bigger breasts.

These symptoms happen because the tumor makes too much hormone. This messes up the body’s hormone balance.

Non-Hormonal Symptoms and Warning Signs

Not all symptoms of adrenal cancer come from hormone changes. Some people might feel:

1. Abdominal pain or discomfort from the tumor’s size.
2. Unexplained weight loss.
3. Fatigue or feeling weak.

These symptoms are not unique to adrenal cancer. They can be signs of many other things. But, when seen with other symptoms, they might point to adrenal cancer.

Spotting these symptoms early is key. Knowing them can help find the cancer sooner. This might lead to better treatment options.

Diagnostic Procedures for Adrenal Cortical Carcinoma

We use a detailed method to find adrenal cortical carcinoma. This includes a physical check, lab tests, and imaging. These steps help us diagnose and understand the disease’s stage.

Initial Assessment and Physical Examination

The first step is a thorough check and physical exam. We look for signs like abdominal masses or hormonal imbalances. We also ask about your medical history to find risk factors.

Laboratory Tests and Hormone Assessments

Labs are key in finding adrenal cortical carcinoma. We test for hormone levels to see if a tumor is working. Other tests look for metabolic issues or other signs of the disease.

Some important tests are:

Imaging Studies and Biopsy Procedures

Imaging is vital to see the adrenal glands and the disease’s spread. We use CT scans, MRI, and PET scans. Sometimes, a biopsy is needed to examine tissue.

By combining the results from these steps, we can accurately diagnose adrenal cortical carcinoma. Then, we create a treatment plan.

Staging and Grading Systems

Staging and grading of adrenal cortical carcinoma are key to predicting patient outcomes. They help guide treatment plans. Accurate staging and grading are essential for understanding the disease’s severity.

TNM Classification System for Adrenal Cancer

The TNM system is a common method for staging adrenal cortical carcinoma. It looks at the tumor’s size (T), nearby lymph nodes (N), and distant metastasis (M). This helps doctors predict the cancer’s spread and choose the best treatment.

TNM Staging: The T category checks the tumor’s size and spread. The N category looks at lymph node involvement. The M category checks for distant metastasis.

Histopathological Grading and Weiss Criteria

Histopathological grading is vital for evaluating adrenal cortical carcinoma. The Weiss criteria help tell benign from malignant tumors. They look at cell shape, mitotic rate, and necrosis.

Weiss Criteria: A score of 3 or more means the tumor is malignant. The Weiss criteria help pathologists understand the tumor’s aggressiveness. This is key for choosing the right treatment.

Using the TNM system and Weiss criteria together gives a full picture of the disease. This helps doctors create a treatment plan that fits each patient’s needs.

Comprehensive Treatment Approaches

Managing adrenal cortical carcinoma requires a team effort. This includes surgery, radiology, and systemic treatments. We focus on creating a treatment plan that fits each patient’s needs.

Surgical Management Options

Surgery is often the first step in treating adrenal cortical carcinoma. Surgical resection aims to remove the tumor completely. This can be a cure if the cancer is caught early.

Our surgeons use both laparoscopic and open surgery. This depends on the tumor’s size and where it is.

A study in the Journal of Clinical Oncology shows surgery improves survival rates. Our team works together to give patients the best care.

Radiation Therapy Applications

Radiation therapy is used when surgery isn’t possible. External beam radiation therapy sends high-energy beams to the tumor. This helps shrink the tumor and ease symptoms.

We use the latest radiation technology to protect healthy tissues. Our radiation oncology team works with surgeons and medical oncologists to plan treatment.

Systemic Treatments

Systemic treatments, like chemotherapy and targeted therapy, are for advanced cases. Mitotane is a key medication that helps control the disease.

We offer the newest systemic treatments and clinical trials. Our medical oncology team creates a treatment plan that meets each patient’s needs and goals.

Advanced Treatments and Clinical Trials

Advanced treatments and clinical trials are key in the fight against Adrenal Cortical Carcinoma. As we learn more about this disease, new treatments are being created. These aim to target specific parts of the cancer.

Emerging Therapeutic Approaches

New research has found targets for Adrenal Cortical Carcinoma. Targeted therapy is showing great promise. It uses drugs to block pathways that help tumors grow.

For example, treatments for the IGF2 pathway are being tested. This is because IGF2 is often too active in many cases of Adrenal Cortical Carcinoma.

“The development of targeted therapies represents a significant shift towards more personalized medicine in the treatment of Adrenal Cortical Carcinoma.”

Immunotherapy Developments

Immunotherapy is a new hope in fighting cancer. It uses the body’s immune system to attack cancer cells. Checkpoint inhibitors, a type of immunotherapy, are being tested for Adrenal Cortical Carcinoma.

“Immunotherapy has revolutionized the treatment landscape for many cancers, including Adrenal Cortical Carcinoma.”

Participating in Clinical Trials

Clinical trials are vital for improving Adrenal Cortical Carcinoma treatments. They offer patients new treatments not yet available. We urge patients to talk to their doctors about joining a trial.

• Discuss eligibility criteria with your healthcare provider.
• Understand the benefits and risks.
• Think about helping medical science advance.

Conclusion

Understanding adrenal cortical carcinoma cancer is key to managing it well. We’ve looked into its complexities, from what it is to how it’s treated.

Dealing with this rare disease needs a team effort. This includes surgery, radiation, and other treatments. Knowing about new treatments helps patients make better choices.

We at our institution aim to give top-notch care to all patients, including those from abroad. We stress the importance of knowing about the disease early. Together, we can help patients get the best care and support they need.

References

National Cancer Institute — Adrenocortical Carcinoma Treatment (PDQ®)

https://www.cancer.gov/types/adrenocortical/patient/adrenocortical-treatment-pdq

VICC — Adult Adrenocortical Carcinoma

https://vicc.org/cancer-info/adult-adrenocortical-carcinoma

American Cancer Society — Adrenal Cancer: Signs, Symptoms, Detection & Diagnosis

https://www.cancer.org/cancer/types/adrenal-cancer/detection-diagnosis-staging/signs-symptoms.html