Adrenocortical Carcinoma Symptoms

Adrenocortical carcinoma (ACC) symptoms often arise from the tumor's size or its production of excess hormones, such as cortisol or androgens. These rare, aggressive cancers of the adrenal glands can cause varied signs, including weight changes, high blood pressure, muscle weakness, or new hair growth. Recognizing these symptoms early is crucial for timely diagnosis and treatment.

What is Adrenocortical Carcinoma Symptoms?

Adrenocortical carcinoma (ACC) is a rare and aggressive cancer that starts in the outer layer of your adrenal glands, which are small glands located on top of each kidney. Symptoms often appear when the tumor grows large enough to press on nearby organs or when it produces too many hormones, leading to various body changes.

Adrenocortical carcinoma is a serious condition because it can grow quickly and spread to other parts of the body. The adrenal glands are vital for producing hormones that regulate many bodily functions, including metabolism, blood pressure, and stress response. When a tumor forms in these glands, it can disrupt normal hormone production. Many people with ACC experience symptoms related to an overproduction of hormones. These are called "functional" tumors. Other tumors, known as "non-functional," do not produce excess hormones. Their symptoms are usually due to the tumor's physical presence and growth within the abdomen. Because ACC is rare, its symptoms can sometimes be mistaken for more common conditions. This can make diagnosis challenging. It is important to pay attention to new or worsening symptoms and discuss them with your doctor, especially if they are persistent or unusual.

Symptoms

Adrenocortical carcinoma symptoms 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), new hair growth, or abdominal pain. These symptoms can develop gradually and may be subtle at first, making them easy to overlook.

Many symptoms of ACC are caused by the tumor making too many hormones. The most common hormone produced in excess is cortisol, leading to symptoms similar to Cushing's syndrome. These can include weight gain, especially in the face and trunk, muscle weakness, easy bruising, and purple stretch marks on the skin. You might also experience high blood pressure (hypertension), high blood sugar (diabetes), and mood changes like anxiety or depression. Some ACC tumors produce too many androgens, which are male hormones. In women, this can lead to new or increased facial and body hair (hirsutism), deepening of the voice, and enlargement of the clitoris. In men, excess androgens are less noticeable, but some might experience breast enlargement (gynecomastia) or testicular shrinkage if the tumor produces estrogens (female hormones). Other hormone imbalances can also occur. For example, excess aldosterone can cause high blood pressure and low potassium levels, leading to muscle weakness and fatigue. When tumors do not produce hormones, symptoms are usually related to the tumor's physical size. These can include a feeling of fullness or a lump in the abdomen, abdominal pain, back pain, unexplained weight loss, and general fatigue. These non-hormonal symptoms often appear later as the tumor grows larger.

Causes & risk factors

The exact cause of adrenocortical carcinoma (ACC) is often unknown, with most cases occurring sporadically without a clear reason. However, certain rare inherited genetic syndromes can increase your risk. These syndromes involve specific gene mutations that make individuals more susceptible to developing various cancers, including ACC.

Most cases of adrenocortical carcinoma are considered sporadic, meaning they happen by chance and are not inherited. Researchers do not fully understand why these tumors develop in most people. They are not typically linked to lifestyle factors or environmental exposures. However, a small number of ACC cases are associated with inherited genetic conditions. These conditions include Li-Fraumeni syndrome, which involves a mutation in the TP53 gene and increases the risk of several cancers. Another is Beckwith-Wiedemann syndrome, a growth disorder that can also predispose individuals to ACC, especially in childhood. Multiple Endocrine Neoplasia type 1 (MEN1) syndrome is another genetic condition linked to an increased risk of ACC. This syndrome affects several endocrine glands, including the adrenal glands. If you have a family history of these syndromes or have been diagnosed with one, your doctor may recommend regular screenings to monitor for potential tumor development.

How it's diagnosed

Diagnosing adrenocortical carcinoma (ACC) typically involves a combination of imaging tests and hormone level assessments. Doctors use scans like CT or MRI to locate the tumor and check its size and spread. Blood and urine tests measure hormone levels to see if the tumor is overproducing them. A biopsy may be performed, often during surgery, to confirm the cancer.

When you visit your doctor with symptoms that suggest ACC, they will likely start with a physical exam and ask about your medical history. The first step in diagnosis often involves imaging tests. A computed tomography (CT) scan or magnetic resonance imaging (MRI) of your abdomen can help locate the adrenal tumor, determine its size, and see if it has spread to nearby tissues or organs. Positron emission tomography (PET) scans may also be used to detect cancer spread throughout the body. Blood and urine tests are crucial for checking hormone levels. Your doctor will look for elevated levels of cortisol, androgens, estrogens, or aldosterone, depending on your symptoms. These tests help determine if the tumor is functional (producing hormones) and can provide clues about the type of adrenal tumor present. For example, a 24-hour urine collection can measure cortisol levels to confirm Cushing's syndrome. A definitive diagnosis of ACC usually requires a tissue sample (biopsy). However, biopsies of adrenal masses are often performed with caution. In some cases, a biopsy might be done after the tumor has been surgically removed, as performing a needle biopsy before surgery carries a small risk of spreading cancer cells. Your medical team will decide the safest and most effective diagnostic approach for your specific situation.

Treatment options

Treatment for adrenocortical carcinoma (ACC) primarily involves surgery to remove the tumor, which offers the best chance for a cure, especially when caught early. Depending on the cancer's stage and spread, additional treatments may include chemotherapy, radiation therapy, or targeted therapies. Your treatment plan will be tailored by a team of specialists to your specific condition.

Surgery is the main treatment for adrenocortical carcinoma, particularly if the cancer has not spread beyond the adrenal gland. The goal of surgery is to remove the entire tumor, along with the affected adrenal gland and any nearby lymph nodes that might contain cancer cells. Complete surgical removal offers the best chance for long-term survival. If the cancer has spread or cannot be entirely removed by surgery, other treatments may be used. Chemotherapy drugs, such as mitotane, are often used to kill cancer cells or slow their growth. Mitotane can be given after surgery to reduce the risk of recurrence or to treat advanced cancer. Other chemotherapy regimens may also be used. Radiation therapy uses high-energy beams to destroy cancer cells or shrink tumors. It may be used after surgery to kill any remaining cancer cells or to relieve symptoms if the cancer has spread to other areas, like bones. Targeted therapies, which focus on specific vulnerabilities in cancer cells, are also being explored and may be an option for some individuals, often in clinical trials. Your treatment team, including oncologists and endocrinologists, will discuss the most appropriate options for you.

Recovery & outlook

The recovery and outlook for adrenocortical carcinoma (ACC) vary significantly, largely depending on the cancer's stage at diagnosis and the completeness of surgical removal. ACC is often an aggressive cancer, and early detection and complete surgical resection offer the best prognosis. Ongoing monitoring is essential after treatment to watch for any recurrence.

Adrenocortical carcinoma is considered an aggressive cancer, and its outlook can be challenging. The most important factor influencing recovery and prognosis is the stage of the cancer when it is diagnosed. Cancers that are small and confined to the adrenal gland (early stage) have a better prognosis than those that have spread to nearby tissues or distant organs (advanced stage). Complete surgical removal of the tumor is critical for a favorable outcome. If the surgeon can remove all visible cancer, the chances of long-term survival improve significantly. However, even after successful surgery, ACC has a tendency to recur, meaning it can come back. This is why regular follow-up appointments, including imaging scans and hormone tests, are vital after treatment. Living with ACC and its treatment can be physically and emotionally demanding. Support groups, counseling, and palliative care can help manage symptoms, side effects, and emotional well-being. While the outlook for advanced ACC can be poor, ongoing research is continually improving treatment options and understanding of the disease, offering hope for better outcomes in the future.

When to see a doctor

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

It is important to consult your doctor if you notice any new or unusual symptoms that persist over time. While many of these symptoms can be caused by less serious conditions, it is always best to have them checked. Pay particular attention to symptoms related to hormone imbalances, such as unexplained weight gain or loss, new or excessive hair growth (hirsutism) in women, or changes in your voice. Seek medical attention if you develop high blood pressure that is difficult to control with medication, or if you experience unexplained muscle weakness or fatigue. Any new abdominal pain, a feeling of fullness, or a palpable lump in your abdomen should also prompt a visit to your doctor. These could be signs of a growing tumor. Do not delay seeking medical advice if you have concerns. Early diagnosis of adrenocortical carcinoma can significantly impact treatment effectiveness and overall prognosis. Your doctor can perform an examination and order appropriate tests to determine the cause of your symptoms and recommend the next steps.

Frequently asked questions

Is adrenocortical carcinoma a common cancer?

Adrenocortical carcinoma (ACC) is a very rare type of cancer. It affects only about 1 to 2 people per million each year. Its rarity means that many doctors may not encounter it frequently, making diagnosis challenging.

Can adrenocortical carcinoma be cured?

A cure for adrenocortical carcinoma is most likely when the cancer is diagnosed at an early stage and can be completely removed through surgery. If the cancer has spread, it becomes more challenging to cure, but treatments can help manage the disease and improve quality of life.

Are adrenocortical carcinoma symptoms always obvious?

No, adrenocortical carcinoma symptoms are not always obvious. They can be subtle and develop gradually, often mimicking symptoms of more common conditions. Some tumors may not produce hormones, causing symptoms only when they grow large enough to press on other organs.

What is the difference between a functional and non-functional adrenocortical carcinoma?

A functional adrenocortical carcinoma produces excess hormones, leading to symptoms like weight gain, high blood pressure, or new hair growth. A non-functional tumor does not produce excess hormones, so its symptoms are usually related to its size, such as abdominal pain or a lump.

Does adrenocortical carcinoma run in families?

Most cases of adrenocortical carcinoma occur sporadically, meaning they are not inherited. However, a small percentage of cases are linked to rare inherited genetic syndromes, such as Li-Fraumeni syndrome or Beckwith-Wiedemann syndrome, which can increase the risk.

What kind of doctor treats adrenocortical carcinoma?

Adrenocortical carcinoma is typically managed by a multidisciplinary team of specialists. This team often includes an endocrinologist (hormone specialist), an oncologist (cancer specialist), a surgeon, and sometimes a radiation oncologist.

Sources

• MedlinePlus — Adrenocortical Carcinoma Symptoms
• Mayo Clinic — Adrenocortical Carcinoma Symptoms
• Cochrane Library — Adrenocortical Carcinoma Symptoms