Last Updated on November 26, 2025 by Bilal Hasdemir
Adrenocortical carcinoma (ACC) is a rare and aggressive cancer. It starts in the adrenal cortex. Getting a diagnosis of adrenal cortical cancer can be very tough.
The adrenal glands sit on top of each kidney. They make hormones that help our bodies work right. ACC can make too many hormones or not make any at all. At Liv Hospital, we offer expert help and care for those with adrenocortical carcinoma.
ACC is very rare, happening in about 1 in 1 million people each year. It needs careful and detailed care.
Key Takeaways
- Adrenocortical carcinoma is a rare and aggressive cancer.
- ACC can be functioning or non-functioning.
- Liv Hospital provides expert guidance and personalized care.
- ACC diagnosis requires thorough care and attention.
- Early detection is key for good treatment.
Understanding Adrenocortical Carcinoma (ACC)
Adrenocortical carcinoma (ACC) is a rare cancer that affects the adrenal glands. These glands are vital for our body’s functions. ACC disrupts how these glands work.
Definition and Overview
ACC is a cancer of the adrenal cortex. This part of the glands makes hormones like cortisol and aldosterone. These hormones help us stay healthy and respond to stress.
ACC is rare and can make too many hormones. It can also grow fast and spread. Research shows that genetics play a big role in ACC. Mutations in genes like TP53 are common in this cancer.
The Adrenal Glands and Their Function
The adrenal glands sit on top of each kidney. They make hormones that control our metabolism, immune system, and blood pressure. The outer part, the cortex, makes corticosteroids and mineralocorticoids. The inner part, the medulla, makes catecholamines.
Our health depends on the adrenal glands working right. Problems, like ACC, can cause big health issues.
Prevalence and Epidemiology
ACC is rare, with about 1-2 cases per million people each year. It can happen at any age but is most common in people between 40 and 50. Some studies show it’s slightly more common in women.
| Characteristics | Description |
|---|---|
| Annual Incidence | 1-2 cases per million |
| Age of Onset | Most common between 40-50 years |
| Gender Predominance | Slightly more common in females |
More research is needed to understand ACC better. This will help find better ways to diagnose and treat it.
Types of Adrenocortical Carcinoma
It’s important to know the different types of adrenocortical carcinoma to find the right treatment. We divide ACC into types based on its function, hormone production, and how far it has spread.
Functioning vs. Non-Functioning Tumors
ACC tumors are either functioning or non-functioning. Functioning tumors make too many hormones, causing certain symptoms. Non-functioning tumors don’t make hormones but can grow big and be dangerous.
Functioning ACCs can make hormones like cortisol, androgens, and estrogens. The hormone made affects how symptoms show up. For example, too much cortisol can cause Cushing’s syndrome, leading to weight gain and high blood pressure.
Hormone-Producing Classifications
ACC is also classified by the hormone it makes. The main types are:
- Cortisol-producing tumors, leading to Cushing’s syndrome
- Androgen-producing tumors, causing virilization
- Estrogen-producing tumors, resulting in feminization
- Tumors producing a combination of hormones
| Hormone Produced | Clinical Syndrome | Symptoms |
|---|---|---|
| Cortisol | Cushing’s Syndrome | Weight gain, hypertension, glucose intolerance |
| Androgens | Virilization | Masculinization in females, precocious puberty in males |
| Estrogens | Feminization | Feminization in males, menstrual irregularities in females |
Localized vs. Metastatic Disease
ACC can also be classified by how far it has spread. Localized disease stays in the adrenal gland. Metastatic disease spreads to other places like the liver or lungs. Knowing this helps decide the best treatment.
Understanding the type and spread of ACC is key to a good treatment plan. We’ll look at treatment options next.
Common Symptoms of Adrenocortical Carcinoma
It’s important to know the symptoms of adrenocortical carcinoma early. This cancer can cause many symptoms because it makes too many hormones. Knowing these symptoms helps in treating the disease early.
Hormone-Related Symptoms
When adrenocortical carcinoma makes too many hormones, it causes certain symptoms. The symptoms depend on the hormone made. For example, too much cortisol can cause Cushing’s syndrome. This includes weight gain, high blood pressure, and changes in skin and hair.
Cushing’s Syndrome Manifestations
Cushing’s syndrome is common in people with this cancer. Symptoms include rapid weight gain in the face and trunk, fatigue, and high blood pressure. Other signs are mood changes and hirsutism (too much hair).
Virilization and Feminization Signs
ACC can also make sex hormones. This can make women look more like men and men look more like women. Signs in women include deepening of the voice, increased body hair, and male pattern baldness. In men, signs include gynecomastia (breast enlargement) and decreased libido.
Non-Specific Symptoms
Not all symptoms are about hormones. Non-specific symptoms include abdominal pain, weight loss, and fatigue. These can be like symptoms of other diseases, making it hard to diagnose.
It’s key to recognize these symptoms to diagnose and treat adrenocortical carcinoma early. Early treatment can greatly improve the patient’s chances of recovery.
Causes and Risk Factors
The exact causes of adrenocortical carcinoma are not fully known. Yet, genetic predispositions and environmental factors are key. We’re studying how these factors lead to this rare cancer.
Genetic Predisposition
Genetic syndromes increase the risk of adrenocortical carcinoma. Li-Fraumeni and Beckwith-Wiedemann syndromes are linked to ACC. These syndromes have mutations in genes that usually prevent cancer.
Li-Fraumeni Syndrome: This rare disorder is caused by TP53 gene mutations. People with it are at higher risk for several cancers, including ACC.
Beckwith-Wiedemann Syndrome: This condition has genetic changes on chromosome 11, affecting the IGF2 gene. It leads to overgrowth and a higher risk of childhood cancer, including ACC.
Tumor Suppressor Genes (TP53, IGF2)
Tumor suppressor genes like TP53 and IGF2 control cell growth and prevent cancer. Mutations in these genes can cause uncontrolled cell growth and tumors. Ongoing research aims to understand their role in ACC.
| Gene | Function | Association with ACC |
|---|---|---|
| TP53 | Tumor suppressor gene, regulates cell division | Mutations associated with Li-Fraumeni syndrome and increased ACC risk |
| IGF2 | Involved in cell growth and development | Overexpression linked to Beckwith-Wiedemann syndrome and ACC |
Environmental Factors
The role of environmental factors in adrenocortical carcinoma is not well understood. Some chemicals and radiation may increase cancer risk. More research is needed to find specific environmental risks for ACC.
Age and Gender Considerations
Adrenocortical carcinoma can happen at any age but is more common in some groups. It’s also more common in women, according to some studies. Knowing these patterns helps identify at-risk groups.
Genetic Syndromes Associated with ACC
Understanding the genetic causes of adrenocortical carcinoma is key to finding those at high risk. Some genetic syndromes are linked to a higher chance of getting this rare and aggressive cancer.
Li-Fraumeni Syndrome
Li-Fraumeni syndrome is a rare genetic disorder. It’s caused by mutations in the TP53 tumor suppressor gene. People with this syndrome are more likely to get several cancers, including adrenocortical carcinoma.
Key Features:
- Early onset of various cancers
- Multiple primary tumors
- Family history of cancer
Beckwith-Wiedemann Syndrome
Beckwith-Wiedemann syndrome is a condition that leads to overgrowth. It increases the risk of childhood cancer, including adrenocortical carcinoma. This condition is linked to chromosome 11 abnormalities.
| Characteristics | Implications |
|---|---|
| Macroglossia | Increased risk of omphalocele and other abdominal wall defects |
| Overgrowth | Increased risk of tumor development |
Multiple Endocrine Neoplasia (MEN1)
MEN1 is a hereditary condition that causes tumors in multiple endocrine glands. It’s mainly linked to parathyroid, pancreatic, and pituitary tumors. But, it can also raise the risk of adrenocortical tumors.
“The genetic basis of MEN1 involves mutations in the MEN1 gene, which acts as a tumor suppressor. Identifying MEN1 mutations can help in the early detection and management of associated tumors.”
Lynch Syndrome
Lynch syndrome, also known as hereditary nonpolyposis colorectal cancer (HNPCC), is mainly linked to colorectal cancer. But, it can also raise the risk of other cancers, including adrenocortical carcinoma.
It’s important to recognize these genetic syndromes for early detection and management of adrenocortical carcinoma. Genetic counseling and testing can help find those at risk. This allows for targeted surveillance and preventive measures.
Diagnosing Adrenal Cortex Carcinoma
To diagnose adrenal cortex carcinoma, we use a detailed approach. This includes imaging studies and lab tests. We will explain how to identify this condition accurately.
Initial Assessment and Physical Examination
The first step is a thorough clinical evaluation and physical exam. We look for signs like abdominal pain, weight changes, or hormonal imbalances. A detailed medical history helps identify risk factors.
During the physical exam, we check for signs of hormonal excess. This can include Cushing’s syndrome or virilization. These signs help us understand the tumor’s nature.
Imaging Studies (CT, MRI, PET Scans)
Imaging studies are key in diagnosing ACC. We use different imaging methods to see the tumor and its characteristics.
- CT Scans: Give detailed images of the adrenal glands. They help find tumors and check their size and spread.
- MRI: Provides high-resolution images. These help tell if a tumor is benign or malignant.
- PET Scans: Detect metastatic disease and check the tumor’s metabolic activity.
Laboratory Tests and Hormone Evaluation
Laboratory tests are vital for checking the tumor’s function and hormone levels. We measure hormones and their metabolites to see if the tumor is producing too much.
| Hormone/Test | Significance |
|---|---|
| Cortisol | Elevated levels may indicate Cushing’s syndrome. |
| Androgens | Excess levels can cause virilization. |
| Aldosterone | High levels may lead to hypertension and hypokalemia. |
“The accurate diagnosis of adrenocortical carcinoma relies heavily on a combination of imaging studies and laboratory tests to assess both the anatomical and functional characteristics of the tumor.”
Biopsy and Pathological Confirmation
A biopsy is often the final step in confirming ACC. We examine the tumor tissue to check its malignancy and characteristics, like the Ki-67 proliferation index.
The pathological examination gives us critical information about the tumor’s aggressiveness. It helps guide treatment decisions.
By combining clinical evaluation, imaging studies, lab tests, and pathological examination, we can accurately diagnose adrenocortical carcinoma. This allows us to develop an effective treatment plan.
Staging and Classification of Adrenocortical Carcinoma
Accurate staging of adrenocortical carcinoma (ACC) is key to predicting patient outcomes. It helps guide clinical decisions. Staging looks at the tumor’s spread and characteristics to determine disease severity.
TNM Staging System
The TNM staging system is a common method for classifying tumors. It checks the tumor’s size and spread, nearby lymph nodes, and distant metastasis. For ACC, it helps sort patients into risk groups, affecting treatment and prognosis.
TNM staging components:
- Tumor (T): Size and extent of the primary tumor
- Node (N): Involvement of regional lymph nodes
- Metastasis (M): Presence of distant metastasis
ENSAT Staging System
The European Network for the Study of Adrenal Tumors (ENSAT) staging is specific to adrenocortical carcinoma. It helps assess disease extent and predict outcomes. The ENSAT system is great for finding patients who might need aggressive or targeted treatments.
Weiss Criteria for Malignancy
The Weiss criteria help tell benign from malignant adrenocortical tumors. They look at tumor size, mitotic rate, and necrosis. Pathologists use these criteria to judge a tumor’s malignancy, aiding in diagnosis and treatment.
Ki-67 Proliferation Index
The Ki-67 index is a marker of cell growth important in ACC. A high index means a more aggressive tumor, linked to worse outcomes. This info is key for treatment decisions and discussing prognosis with patients.
Using these staging and classification systems together helps us understand ACC better. We can then create personalized treatment plans for each patient’s unique needs.
Treatment Approaches for Adrenal Carcinoma Cancer
Dealing with adrenocortical carcinoma cancer needs a detailed plan. This plan often includes several treatments. The right treatment depends on the tumor’s stage, its type, and the patient’s health.
Surgical Management
Surgery is key for treating adrenocortical carcinoma that’s just starting. The main goal is to take out the tumor and any nearby affected tissues. We use modern surgery methods to cut down on recovery time and improve results.
At times, surgery might be followed or come before other treatments. These could be chemotherapy or radiation therapy to get rid of any cancer cells left behind.
Radiation Therapy
Radiation therapy is used in specific cases. This could be when the tumor can’t be fully removed or has spread. This treatment helps slow down tumor growth and ease symptoms. We use advanced radiation methods like IMRT to precisely target the tumor and protect healthy tissues.
Chemotherapy Options
Chemotherapy is a common treatment for advanced or spread-out adrenocortical carcinoma. The right chemotherapy depends on the tumor and the patient’s health. We look at different chemotherapy options to get the best results.
Mitotane and Adrenal-Specific Medications
Mitotane is a special medication for adrenocortical carcinoma. This drug lowers adrenal hormone production and can help some patients. We watch patients on Mitotane closely to manage side effects and adjust the dose as needed.
It’s vital to have a team approach for treating adrenocortical carcinoma. Our team creates a custom treatment plan. We use the latest in surgery, medicine, and radiation to help patients the most.
Living with Adrenocortical Carcinoma
Living with adrenocortical carcinoma (ACC) means we need a full plan to manage it well. We must tackle the tumor, hormonal imbalances, and emotional hurdles it brings.
Managing Hormone Imbalances
One big challenge with ACC is handling hormone imbalances. Tumors can make too much of certain hormones. This can cause weight gain, high blood pressure, and mood swings.
We must work with doctors to keep an eye on hormone levels. This helps us adjust treatments as needed.
Medicines like mitotane can help control hormone levels. Regular blood tests and scans are key to seeing if treatments are working.
Coping with Treatment Side Effects
Treatment for ACC can affect a person’s life a lot. Surgery, chemo, and radiation each have their own side effects. It’s important to know these and find ways to lessen them.
For example, chemo can make you feel sick, tired, and lose your hair. But, there are ways to help with these side effects, like anti-nausea meds and eating right.
Supportive Care and Resources
It’s not just about fighting the cancer. We also need to take care of our overall health. Supportive care is key here. It includes mental support, nutrition advice, and pain relief.
“The emotional and psychological impact of adrenocortical carcinoma should not be underestimated. Support groups and counseling can provide invaluable support to patients and their families.” –
Support groups, online or in-person, are great. They connect people with others facing similar challenges. This brings a sense of community and understanding.
Follow-up Care and Monitoring
Regular check-ups are vital to catch any signs of cancer coming back early. We recommend sticking to a schedule of tests and scans.
| Follow-up Procedure | Frequency | Purpose |
|---|---|---|
| Imaging Studies (CT, MRI) | Every 3-6 months | Monitor for recurrence or metastasis |
| Blood Tests (hormone levels, tumor markers) | Every 3-6 months | Assess hormone production and tumor activity |
By being proactive and vigilant, we can make life better for those with adrenocortical carcinoma.
Conclusion
Understanding adrenocortical carcinoma (ACC) is key for early detection and effective treatment. We’ve looked into the complexities of this rare cancer. This includes its types, symptoms, and treatment options.
Diagnosing ACC requires a detailed approach. This includes imaging studies, lab tests, and pathological confirmation. The stage and type of ACC help determine the prognosis and treatment plan.
New treatments for ACC have improved patient outcomes. Surgical, radiation, and chemotherapy are used. Also, medications like mitotane show promise in managing the disease.
The prognosis for adrenal carcinoma varies based on several factors. Ongoing research and new treatments offer hope for better care and outcomes. As we learn more about ACC, we can offer more effective support and treatments.
FAQ
What is adrenocortical carcinoma (ACC), and how does it differ from other types of adrenal gland cancers?
Adrenocortical carcinoma (ACC) is a rare and aggressive cancer. It starts in the outer layer of the adrenal gland. Unlike other adrenal gland cancers, it can produce too much hormone.
What are the symptoms of adrenocortical carcinoma, and how do they vary depending on the type of tumor?
Symptoms of ACC vary based on the tumor type. Functioning tumors can lead to Cushing’s syndrome or changes in sex characteristics. Non-functioning tumors may cause pain or weight loss without hormone changes.
What are the risk factors for developing adrenocortical carcinoma, and how do genetic syndromes play a role?
Risk factors for ACC include genetic predisposition and certain syndromes. Li-Fraumeni and Beckwith-Wiedemann syndromes are examples. Mutations in genes like TP53 and IGF2 also increase risk. Age and gender can also play a part.
How is adrenocortical carcinoma diagnosed, and what diagnostic tests are used?
Diagnosing ACC involves several steps. First, a doctor will evaluate you clinically. Then, imaging tests like CT, MRI, and PET scans are used. Hormone level tests and a biopsy are also necessary to confirm the diagnosis.
What are the treatment options for adrenocortical carcinoma, and how are they determined?
Treatment for ACC depends on the tumor’s stage and type. Surgery is often the first choice for early-stage tumors. For more advanced cases, radiation, chemotherapy, or medication like Mitotane may be used.
What is the prognosis for patients with adrenocortical carcinoma, and how does staging impact treatment outcomes?
The prognosis for ACC patients varies based on the tumor’s stage and characteristics. Staging systems like TNM and ENSAT help plan treatment. The Weiss criteria and Ki-67 index also provide important information about the tumor.
How can patients with adrenocortical carcinoma manage hormone imbalances and treatment side effects?
Managing hormone imbalances and side effects is key for ACC patients. Supportive care and resources are vital. This includes hormone replacement therapy and managing side effects to improve quality of life.
What is the role of follow-up care and monitoring in adrenocortical carcinoma management?
Regular follow-up and monitoring are critical. They help catch recurrence or metastasis early. This allows for timely adjustments to treatment plans.
Are there any ongoing research or advancements in the treatment of adrenocortical carcinoma?
Yes, research is ongoing. New treatments like targeted therapies and immunotherapies are being explored. These advancements aim to improve care and outcomes for ACC patients.
References
American Cancer Society (Cancer.org): What Is Adrenal Cancer?
Medical News Today: Adrenocortical Carcinoma
