Living With Adrenocortical Carcinoma

Living with adrenocortical carcinoma (ACC) means managing a rare and aggressive cancer that starts in the outer layer of the adrenal glands. It often involves ongoing medical care, including treatments like surgery, chemotherapy, or radiation, and regular monitoring. People living with ACC focus on managing symptoms, treatment side effects, and maintaining their quality of life with support from their healthcare team.

What is Living With Adrenocortical Carcinoma?

Living with adrenocortical carcinoma (ACC) means navigating the challenges of a rare cancer that develops in the adrenal glands, which are small glands located above your kidneys. This journey often involves managing symptoms caused by hormone imbalances, undergoing various treatments, and participating in regular follow-up care. Support from a specialized medical team is crucial for managing the condition and its impact on daily life.

Adrenocortical carcinoma is a very uncommon type of cancer. It forms in the adrenal cortex, the outer part of your adrenal glands. These glands make important hormones that control many body functions, such as blood pressure, metabolism, and how your body responds to stress. When ACC develops, it can produce too many hormones, leading to various symptoms, or it can grow large enough to cause problems by pressing on other organs. Because ACC is rare, it is often managed by a team of specialists. This team may include endocrinologists (hormone specialists), oncologists (cancer specialists), and surgeons. They work together to create a personalized treatment plan. Living with ACC requires ongoing medical attention and a focus on managing both the cancer itself and any related symptoms or treatment side effects.

Symptoms

Symptoms of adrenocortical carcinoma (ACC) can vary widely, often depending on whether the tumor produces excess hormones or its size. Common signs include weight changes, muscle weakness, high blood pressure (hypertension), and new hair growth or changes in menstrual cycles. It is important to discuss any new or worsening symptoms with your doctor, as early detection can be key.

Many symptoms of ACC are caused by the tumor making too many hormones. For example, if the tumor produces too much cortisol, you might experience symptoms similar to Cushing's syndrome. These can include weight gain, especially around the face and trunk, easy bruising, muscle weakness, and high blood sugar (diabetes). You might also notice a rounded face (moon face) or a hump between your shoulders (buffalo hump). If the tumor makes too many male hormones (androgens), women might notice increased facial or body hair (hirsutism), acne, or a deepening voice. Men might experience breast enlargement (gynecomastia) if the tumor produces too much estrogen. Other general symptoms, regardless of hormone production, can include pain in the abdomen or back, a feeling of fullness, or a lump that can be felt in the abdomen. These symptoms often develop slowly over time.

Causes & risk factors

The exact causes of adrenocortical carcinoma (ACC) are often unknown, meaning it can develop without clear reasons in most people. However, some genetic conditions, such as Li-Fraumeni syndrome or Beckwith-Wiedemann syndrome, can increase a person's risk. Understanding these potential links helps doctors assess individual risk, though ACC remains a very rare cancer overall.

For most people diagnosed with ACC, there is no clear cause or specific risk factor identified. The cancer appears to develop spontaneously. This means that changes in the adrenal gland cells happen without a known trigger. However, certain inherited genetic syndromes are linked to a higher risk of developing ACC. These include Li-Fraumeni syndrome, which increases the risk of several types of cancer, and Beckwith-Wiedemann syndrome, a growth disorder. If you have a family history of these syndromes or ACC, your doctor might recommend genetic counseling or screening. It is important to remember that even with these risk factors, ACC is still a very rare condition.

How it's diagnosed

Diagnosing adrenocortical carcinoma (ACC) typically involves a combination of tests to identify the tumor and determine if it is cancerous. Doctors often start with blood and urine tests to check hormone levels, followed by imaging scans like CT (computed tomography) or MRI (magnetic resonance imaging) to locate the tumor. A biopsy, where a small tissue sample is taken, is usually needed to confirm the diagnosis.

The diagnostic process for ACC usually begins when a person experiences symptoms or when an adrenal mass is found incidentally during imaging for another condition. Initial tests often include blood and 24-hour urine collections to measure levels of various adrenal hormones. High or abnormal hormone levels can suggest an adrenal tumor. If hormone tests are abnormal, or if an adrenal mass is seen, imaging studies are performed. A CT scan or MRI of the abdomen can help determine the size, shape, and location of the tumor. These scans can also show if the tumor has spread to other parts of the body. In some cases, a PET (positron emission tomography) scan may also be used. Finally, a biopsy, which involves taking a small sample of the tumor tissue, is often necessary to confirm if the mass is cancerous (malignant) and to distinguish ACC from non-cancerous (benign) adrenal tumors. This tissue is then examined under a microscope by a pathologist.

Treatment options

Treatment for adrenocortical carcinoma (ACC) depends on the cancer's stage, size, and whether it has spread. Surgery to remove the tumor is often the primary treatment, especially for localized cancer. Other options may include chemotherapy, using drugs to kill cancer cells, or radiation therapy, which uses high-energy rays. Targeted therapies, which focus on specific cancer cell features, are also sometimes used.

The main goal of ACC treatment is to remove as much of the cancer as possible. For early-stage ACC that has not spread, surgery (adrenalectomy) to remove the affected adrenal gland and surrounding tissue is usually the first and most effective treatment. Surgeons aim to remove the entire tumor with clear margins, meaning no cancer cells are found at the edges of the removed tissue. After surgery, or if the cancer has spread, other treatments may be necessary. Chemotherapy, often using a drug called mitotane, is a common treatment for ACC. Mitotane works by destroying adrenal cancer cells and can also help reduce hormone production. Radiation therapy, which uses high-energy X-rays or other types of radiation, may be used to kill cancer cells or to relieve symptoms like pain. In some cases, targeted therapies that block specific pathways involved in cancer growth are considered. Your medical team will discuss the best treatment plan for your specific situation.

Recovery & outlook

Recovery and outlook for adrenocortical carcinoma (ACC) vary greatly among individuals, depending on factors like the cancer's stage at diagnosis and how well it responds to treatment. ACC is often aggressive, making long-term follow-up care essential. Managing treatment side effects, maintaining quality of life, and emotional support are key aspects of living with ACC, with ongoing monitoring for recurrence.

Adrenocortical carcinoma is known to be an aggressive cancer, and the outlook can be challenging. The stage of the cancer at the time of diagnosis is a major factor in determining the prognosis. Cancers that are found early and can be completely removed by surgery generally have a better outlook. However, ACC can recur (come back) even after successful surgery, making regular follow-up appointments crucial. Living with ACC involves continuous monitoring, which includes regular imaging scans and blood tests to check for any signs of recurrence or new tumor growth. Managing side effects from treatments like chemotherapy can also be a significant part of recovery. Many people benefit from support groups, counseling, or palliative care services to help manage symptoms, improve quality of life, and address emotional and psychological challenges. Your healthcare team will work with you to develop a long-term care plan.

When to see a doctor

You should see a doctor if you experience any new or worsening symptoms that could be related to adrenocortical carcinoma (ACC) or its treatments. This includes unexplained weight changes, persistent pain, changes in body hair, or unusual fatigue. Promptly report any severe side effects from medication, such as extreme nausea or dizziness, or signs of infection, as these may require immediate medical attention.

It is important to maintain open communication with your healthcare team when living with ACC. You should contact your doctor if you notice any new symptoms or if existing symptoms become worse. For example, report any new abdominal pain, swelling, or a lump you can feel. Also, be aware of changes in your hormone-related symptoms, such as unexpected weight gain or loss, changes in your menstrual cycle, or new hair growth. Additionally, if you are undergoing treatment for ACC, it is vital to report any severe or concerning side effects immediately. This could include severe nausea, vomiting, fever, chills, unusual bleeding or bruising, or signs of an allergic reaction. Do not wait for your next scheduled appointment if you experience these types of urgent issues. Your medical team can advise you on whether you need to seek emergency care or if they can manage the issue during office hours.

Frequently asked questions

How does adrenocortical carcinoma affect my daily energy levels and ability to work?

Adrenocortical carcinoma (ACC) and its treatments can significantly impact your energy levels, often causing fatigue. Hormone imbalances from the tumor or side effects from chemotherapy can lead to tiredness, muscle weakness, and difficulty concentrating. Many people find they need to adjust their work schedule or daily activities to manage their energy. Discuss strategies for managing fatigue with your healthcare team.

What kind of diet changes might I need to make while living with ACC?

While there isn't a specific "ACC diet," managing symptoms and treatment side effects often involves dietary adjustments. For example, if you have high blood sugar due to excess cortisol, your doctor might recommend a diet to help control it. Nausea from chemotherapy might require bland foods. Eating a balanced diet rich in fruits, vegetables, and lean proteins can support overall health. Always consult your doctor or a dietitian for personalized dietary advice.

Is it safe for me to exercise with adrenocortical carcinoma, and what types of activities are recommended?

Regular, moderate exercise can be beneficial for many people living with adrenocortical carcinoma (ACC), helping to manage fatigue, improve mood, and maintain muscle strength. However, the type and intensity of exercise should be discussed with your healthcare team. They can advise you based on your current health, treatment plan, and any specific limitations, such as bone weakness or surgical recovery. Gentle activities like walking, swimming, or yoga are often good starting points.

How often will I need follow-up appointments and tests after my initial treatment for ACC?

After initial treatment for adrenocortical carcinoma (ACC), regular and frequent follow-up appointments are crucial due to the cancer's aggressive nature and risk of recurrence. Your healthcare team will typically schedule regular physical exams, blood tests to check hormone levels and tumor markers, and imaging scans (like CT or MRI) every few months, especially in the first few years. The frequency may decrease over time if you remain stable, but lifelong monitoring is common.

What emotional support resources are available for people living with adrenocortical carcinoma?

Living with a rare and serious condition like adrenocortical carcinoma (ACC) can be emotionally challenging. Many resources are available to help. These include cancer support groups, where you can connect with others facing similar experiences, and professional counseling or therapy. Your hospital or cancer center may offer social work services that can connect you to local and national support organizations. Openly discussing your feelings with your medical team is also important.

Can adrenocortical carcinoma affect my fertility or ability to have children in the future?

Adrenocortical carcinoma (ACC) and its treatments can potentially affect fertility. Hormone imbalances caused by the tumor can disrupt reproductive cycles. Chemotherapy, especially certain drugs, can also impact fertility in both men and women. If you are concerned about fertility, it is important to discuss this with your healthcare team before starting treatment. They can provide information on potential risks and discuss options for fertility preservation, if appropriate for your situation.

Sources

• MedlinePlus — Living With Adrenocortical Carcinoma
• Mayo Clinic — Living With Adrenocortical Carcinoma
• Cochrane Library — Living With Adrenocortical Carcinoma