Adrenocortical Carcinoma Treatment Options

Adrenocortical carcinoma (ACC) treatment options aim to remove the cancer, stop its growth, and manage symptoms. Treatment usually involves surgery, often followed by medication like mitotane, radiation therapy, or other chemotherapy. The best approach depends on the cancer's stage, your overall health, and specific tumor characteristics, guided by a team of specialists.

What is Adrenocortical Carcinoma Treatment Options?

Adrenocortical carcinoma (ACC) treatment options focus on removing the cancer, slowing its growth, and managing any symptoms it causes. Treatment plans are highly individualized, typically involving surgery, medication (like mitotane), radiation therapy, or a combination of these. A team of medical specialists works together to determine the most effective strategy for each person.

Adrenocortical carcinoma is a rare and aggressive cancer that starts in the outer layer of the adrenal glands (small glands above your kidneys). Because it is rare, treatment often requires a team of experts, including surgeons, oncologists (cancer doctors), and endocrinologists (hormone specialists). This multidisciplinary approach ensures all aspects of the disease are addressed. The main goals of treatment are to remove the tumor if possible, prevent its spread, and control any excess hormone production that may be causing symptoms. The specific treatment chosen depends on several factors. These include the cancer's stage (how far it has spread), whether the tumor can be completely removed, your overall health, and the tumor's specific characteristics.

Symptoms

Symptoms of adrenocortical carcinoma often result from the tumor producing too many hormones or from the tumor growing large enough to press on nearby organs. Common signs include unexplained weight changes, muscle weakness, high blood pressure (hypertension), and abdominal pain or a noticeable lump. These symptoms can vary widely among individuals.

Many symptoms of adrenocortical carcinoma are caused by the adrenal gland making too much of certain hormones. For example, too much cortisol can lead to weight gain, especially around the face and trunk, muscle weakness, and high blood pressure (hypertension). It can also cause high blood sugar (diabetes) and easy bruising. If the tumor produces too many male hormones (androgens), women may experience excess hair growth (hirsutism), a deepening voice, or menstrual irregularities. In rare cases, men might develop enlarged breasts (gynecomastia) if the tumor produces too many female hormones (estrogens). As the tumor grows, it can cause a feeling of fullness or pain in the abdomen, or you might even feel a lump.

Causes & risk factors

The exact cause of most adrenocortical carcinoma cases is unknown, meaning they occur without a clear reason. However, a small number of cases are linked to certain inherited genetic conditions. These conditions increase a person's risk of developing various cancers, including adrenocortical carcinoma, but most people with ACC do not have these syndromes.

Adrenocortical carcinoma is a very rare cancer, affecting about 1 in 1 million people each year in the United States. Most cases are considered "sporadic," meaning they happen by chance and are not inherited. Researchers are still working to understand why these sporadic cases develop. A small percentage of people with adrenocortical carcinoma have an underlying genetic syndrome. Examples include Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and multiple endocrine neoplasia type 1 (MEN1). These syndromes involve specific gene changes that make a person more likely to develop certain tumors. If there is a family history of ACC or related cancers, genetic counseling might be recommended.

How it's diagnosed

Diagnosing adrenocortical carcinoma involves a combination of tests to identify the tumor and determine its nature. Doctors typically use blood and urine tests to check hormone levels, imaging scans like CT or MRI to locate the tumor, and often a biopsy to examine a tissue sample under a microscope for definitive confirmation of cancer.

The diagnostic process usually begins with a physical exam and a review of your symptoms. To check for hormone imbalances, your doctor will order blood and urine tests. These tests measure levels of hormones such as cortisol, aldosterone, and androgens, which can be abnormally high in people with ACC. For example, a 24-hour urine collection can measure cortisol levels. Imaging tests are crucial for finding the tumor and seeing if it has spread. These include computed tomography (CT) scans, magnetic resonance imaging (MRI) scans, and sometimes positron emission tomography (PET) scans. These scans help doctors determine the tumor's size, location, and whether it has invaded nearby tissues or spread to distant parts of the body. A definitive diagnosis of adrenocortical carcinoma usually requires a biopsy, where a small tissue sample is taken from the tumor and examined by a pathologist.

Treatment options

Treatment for adrenocortical carcinoma often involves a combination of approaches tailored to the individual. The primary treatment is usually surgery to remove the tumor. Other key treatments include chemotherapy, often with the drug mitotane, radiation therapy, and sometimes targeted therapies or hormone-blocking medications to manage specific symptoms.

**Surgery:** For many people, surgery is the first and most important treatment for adrenocortical carcinoma, especially if the cancer has not spread beyond the adrenal gland. The goal is to remove the entire tumor (complete resection) and any nearby affected tissue. If the tumor is completely removed, it offers the best chance for a cure or long-term control of the disease. Even if the tumor cannot be fully removed, surgery may be performed to reduce its size and relieve symptoms. **Medication (Chemotherapy):** Mitotane is a specialized chemotherapy drug often used for adrenocortical carcinoma. It works by suppressing the function of the adrenal gland and directly killing cancer cells. Mitotane may be given after surgery (adjuvant therapy) to reduce the risk of the cancer coming back, or it can be used for advanced cancer that has spread. Other chemotherapy drugs, such as cisplatin, doxorubicin, etoposide, and streptozocin, may be used alone or in combination (e.g., the EDP regimen) for more advanced or aggressive disease. **Radiation Therapy:** This treatment uses high-energy X-rays or other particles to kill cancer cells or shrink tumors. Radiation therapy may be used after surgery to destroy any remaining cancer cells, or it can be used to relieve symptoms like pain if the cancer has spread to bones or other areas. It is often delivered externally, aiming beams at the tumor site. **Targeted Therapy and Hormone-Blocking Drugs:** Targeted therapies are newer drugs that focus on specific weaknesses within cancer cells, aiming to block their growth and spread while minimizing harm to healthy cells. These are often explored in clinical trials. Additionally, if the tumor is producing excess hormones, specific hormone-blocking medications can be prescribed to manage these symptoms and improve quality of life.

Recovery & outlook

The recovery and outlook for adrenocortical carcinoma vary significantly, depending mainly on the cancer's stage at diagnosis and how completely the tumor can be removed. Early detection and complete surgical removal offer the best prognosis. Ongoing monitoring is crucial after treatment to watch for any signs of recurrence and manage long-term effects.

After treatment for adrenocortical carcinoma, regular follow-up care is essential. This typically involves frequent physical exams, blood tests to check hormone levels and tumor markers, and imaging scans (like CT or MRI) to monitor for any signs of the cancer returning (recurrence). The frequency of these appointments will be determined by your medical team based on your specific case. The outlook for people with adrenocortical carcinoma is highly individual. When the cancer is found early and completely removed by surgery, the chances of a good outcome are higher. However, ACC can be aggressive, and recurrence is a concern even after successful initial treatment. For advanced cases, treatment focuses on controlling the disease, managing symptoms, and improving quality of life. Clinical trials offer access to new and experimental treatments that may provide additional options.

When to see a doctor

You should see a doctor if you experience new or persistent symptoms that could suggest adrenocortical carcinoma, especially if they are unexplained. These include sudden weight changes, new high blood pressure, unusual hair growth, muscle weakness, or a noticeable lump or persistent pain in your abdomen. Early evaluation is important for any concerning symptoms.

While many of these symptoms can be caused by less serious conditions, it is always wise to consult a healthcare professional if you notice them. For example, if you develop unexplained weight gain or loss, new or worsening high blood pressure (hypertension), or significant muscle weakness, these could be signs of hormone imbalances. Women should pay attention to new facial hair growth, a deepening voice, or irregular periods, which could indicate excess male hormones. Men might notice breast enlargement. Any persistent abdominal pain, a feeling of fullness, or a lump you can feel in your abdomen warrants prompt medical attention. Discussing these symptoms with your doctor allows for timely evaluation and diagnosis.

Frequently asked questions

What is the main treatment for adrenocortical carcinoma?

The main treatment for adrenocortical carcinoma, especially when it hasn't spread, is surgery. The goal of surgery is to remove the entire tumor and any affected surrounding tissue. If the tumor can be completely removed, it offers the best chance for a cure or long-term control of the disease.

What is mitotane, and how does it work for ACC?

Mitotane is a specific chemotherapy drug often used for adrenocortical carcinoma. It works in two main ways: by suppressing the adrenal gland's ability to produce hormones and by directly killing cancer cells. It can be used after surgery to reduce the risk of recurrence or for advanced cancer.

Can adrenocortical carcinoma be cured?

A cure for adrenocortical carcinoma is most likely when the cancer is detected early and completely removed through surgery. For more advanced cases, treatment focuses on controlling the disease's growth, managing symptoms, and improving quality of life, rather than a complete cure.

Are there new treatments for ACC?

Yes, research is ongoing for new treatments for adrenocortical carcinoma. This includes exploring targeted therapies, which aim to attack specific weaknesses in cancer cells, and immunotherapies, which boost the body's own immune system to fight the cancer. These newer treatments are often available through clinical trials.

What are the side effects of ACC treatments?

Side effects vary depending on the treatment. Surgery carries risks like bleeding, infection, or pain. Mitotane commonly causes digestive issues (nausea, vomiting, diarrhea), fatigue, and neurological side effects. Radiation therapy can lead to skin changes, fatigue, and hair loss in the treated area. Your medical team will discuss potential side effects and how to manage them.

How often do I need follow-up after ACC treatment?

Regular follow-up after adrenocortical carcinoma treatment is crucial. It typically involves frequent physical exams, blood tests to check hormone levels and tumor markers, and imaging scans (like CT or MRI). The exact schedule depends on your individual case, the stage of your cancer, and the treatments you received, but it's often ongoing for many years.

Sources

• MedlinePlus — Adrenocortical Carcinoma Treatment Options
• Mayo Clinic — Adrenocortical Carcinoma Treatment Options
• Cochrane Library — Adrenocortical Carcinoma Treatment Options