Adrenocortical Carcinoma: Causes, Symptoms, Diagnosis And Treatment

Introduction

Diagnosed with adrenocortical carcinoma, or ACC? We are here to help you understand what it is and what to expect. ACC is a rare type of cancer that starts in the adrenal glands, those small glands sitting right on top of your kidneys. It’s uncommon, affecting just one or two people out of a million each year. This article provides a detailed explanation of ACC, including its diagnosis and management. Understanding the rarity and intricacies of this condition is also crucial for improving the lives of those affected.

What Is Adrenocortical Carcinoma?

Adrenocortical carcinoma (ACC) is a rare and aggressive form of cancer that initially starts in the outer layer of the adrenal glands, the small hormone-secreting glands that rest atop your kidneys. The glands help keep your body in balance by producing vital hormones. With ACC, the cells within that outer layer begin to grow uncontrollably, and this can result in excess hormones being secreted, which brings about a variety of symptoms and complications. Unfortunately, ACC is often diagnosed when it is already quite advanced, which can make the treatment more complex.

What Are the Types of Adrenocortical Carcinoma?

There are two kinds of these tumors, functioning and non-functioning, and they produce different symptoms:

• Functioning Tumors: These are the more typical type. They overproduce some of the body's hormones, such as aldosterone, cortisol, estrogen, or testosterone. The symptoms depend on which hormone is being secreted.

• Non-functioning Tumors: These do not interfere with hormone levels, but they can become large enough to put pressure on organs and cause pain.

What Are the Causes of Adrenocortical Carcinoma?

Ever wondered what exactly causes adrenocortical carcinoma? While the exact reasons aren’t completely understood, doctors have recognized various risk factors and potential contributors.

Here is what might increase your risk of developing ACC:

1. Genetic Factors: There are some cases of ACC that may be linked to genetic syndromes. Like individuals with Li-Fraumeni syndrome (an inherited cancer risk disorder), or multiple endocrine neoplasia type 1 (MEN1) (a hormone-producing tumor syndrome), or Beckwith-Wiedemann syndrome (an overgrowth genetic disorder), are at a higher risk of developing ACC.

2. Irradiation Exposure: Exposure to radiation, particularly during childhood, is associated with an increased risk of ACC.

3. Age: ACC can occur at any age, but it is most commonly diagnosed in adults; the highest frequency of occurrence is typically seen during individuals' fourth and fifth decades of life.

4. Gender: ACC affects both males and females, but it is slightly more common in women.

5. Hormonal Factors: ACC may be associated with the overproduction of certain hormones. Some hormone-producing tumors in the adrenal cortex, such as those that produce excess cortisol or aldosterone, can be associated with ACC.

6. Inherited Genetic Mutations: Certain inherited genetic mutations can increase the risk of ACC. These mutations may be passed down through families.

What Are the Common Symptoms of Adrenocortical Carcinoma?

The symptoms of ACC depend on whether the tumor is functioning or non-functioning. Some of the common symptoms include:

1. Cushing's Syndrome: Overcortisol production can lead to weight gain, high blood pressure, a rounded face, and muscle weakness.

2. Conn's Syndrome: When too much aldosterone is made, it can lead to high blood pressure, low potassium levels, and muscle cramps.

3. Virilization: Growth in the production of androgens (male hormones) causes masculinization symptoms in women, like a deep voice, body hair, and irregular menstruation.

Non-functioning Tumor:

• Abdominal Pain: There are many non-functioning ACCs that cause pain in the abdomen or back.

• Unexplained Weight Loss: Fast, sudden weight loss can be a symptom of ACC.

• Mass Effect: The size of the tumor can be a palpable mass in the abdomen.

General Symptoms (with or without Hormone Production):

• Fatigue: ACC can cause recurring tiredness.

• Fever: Some people experience unexplained fevers.

• Nausea and Vomiting: As the tumor grows and presses on nearby organs, these symptoms can start to show up.

• Anorexia: Loss of appetite may occur.

How Is Adrenocortical Carcinoma Diagnosed?

Diagnosing adrenocortical carcinoma involves a few key steps in which your doctor will talk to you about your medical history, conduct a physical check-up, recommend some scans and lab tests, and sometimes may even take a small tissue sample.

Here’s a closer look at the diagnostic tests that are involved:

1. Medical History and Physical Examination: The doctor asks your symptoms, past medical history, and any family history of health issues. They will also do a physical exam to look for signs of ACC, like any lumps in your belly or noticeable changes in how you look.

2. Blood and Urine Tests: ACC is associated with some genetic disorders. People suffering from syndromes such as Beckwith-Wiedemann, Li-Fraumeni, or multiple endocrine neoplasia type 1 (MEN1) are at risk of this cancer.

3. Imaging Studies: There are different imaging tests that are used to check the adrenal glands and any tumors present. It includes:

• A CT (computed tomography) scan of the abdomen and pelvis can give you detailed images of the adrenal glands and any tumors.

• MRI (magnetic resonance imaging) may be used to provide additional information about the tumor's size and location.

• A PET (positron emission tomography) scan is useful for ascertaining whether the cancer has metastasized to other areas of the body.

4. Biopsy: A tissue sample of the tumor is often necessary to definitively diagnose ACC. However, biopsies are not performed unless there is a high suspicion of ACC, as they can carry risks of tumor spread. Biopsies are done using image-guided techniques, such as CT or ultrasound, to target the tumor.

5. Genetic Testing: Genetic testing is sometimes suggested, especially if there’s a family history of ACC or related genetic conditions.

6. Staging: Once ACC is confirmed, doctors will figure out how aggressive it is and whether it has spread to other parts of the body. This helps guide the best treatment plan moving forward.

7. Additional Tests: Additional tests, such as adrenocorticotropic hormone (ACTH) stimulation tests or dexamethasone suppression tests, may be performed to assess the tumor's hormone production. ACC is often diagnosed at advanced stages, a rare cancer with variable symptoms.

What Are the Stages of Adrenocortical Carcinoma?

Doctors use something known as cancer staging to know how to treat the cancer most effectively and to gain an idea of what may occur afterwards. In the case of adrenocortical carcinoma, the stage is determined by how large the tumor is, where it is found, and whether or not it has spread to the lymph nodes or other areas of the body.

Here is a brief description of the stages:

• Stage 1: The tumor is approximately two inches or less and is confined to the adrenal gland.

• Stage 2: The cancer is greater than 2 inches but has not spread outside the adrenal gland.

• Stage 3: The cancer has spread nearby the lymph nodes.

• Stage 4: The cancer has reached nearby lymph nodes, adjacent organs, or even farther away within the body.

What Are the Complications of Adrenocortical Carcinoma?

It's completely natural to worry about complications or side effects from ACC treatments, whether it is surgery or the medications prescribed. For instance, if you have surgery to remove an adrenal gland, you might wonder what to expect. Here are a couple of things that could happen, and what you can prepare for:

• Changes in Hormones: More often than not, the remaining adrenal gland takes over and produces enough hormones independently. However, if it does not immediately, you may be required to take hormone tablets for some time until it catches up.

• Infection: There are chances of developing an infection where the procedure took place or in the stomach.

• Bleeding: A few individuals might have heavy bleeding throughout or after the procedure.

How to Treat Adrenocortical Carcinoma?

The treatment of adrenocortical carcinoma often involves a combination of approaches, and the treatment plan is based on the stage of the cancer, the extent of spread, the overall health of the patient, and other factors.

Treatment options for ACC include:

1. Surgery: The main treatment for ACC is the surgical removal of the tumor, an adrenalectomy (surgical removal of one or both adrenal glands). If at all possible, the surgeon attempts to remove the entire tumor. In some cases, they may also need to remove surrounding tissue or lymph nodes. Depending on the situation, the surgery can be performed with minimally invasive techniques such as laparoscopy (minimally invasive surgical procedure) or traditional open surgery.

2. Chemotherapy: Chemotherapy is occasionally utilized as adjuvant therapy (post-surgery) to eradicate any residual cancer cells or as palliative care to alleviate symptoms in cases where ACC has metastasized. Mitotane is a chemotherapy drug commonly used for ACC.

3. Radiation Therapy: Radiation therapy after surgery (adjuvant radiation) is employed to target any remaining cancer cells. It can also be used as palliative therapy to alleviate symptoms or manage pain.

4. Targeted Therapies: Newer treatments called targeted therapies may be used for advanced or metastatic ACC.

5. Immunotherapy: Immunotherapy is an evolving approach that harnesses the body's immune system to fight cancer. While not yet a standard treatment for ACC, clinical trials are trying to investigate the potential of immunotherapy in managing this cancer.

6. Hormone Replacement: After surgery to remove one adrenal gland, some patients may need hormone replacement therapy to replace the missing hormones and maintain hormonal balance.

7. Clinical Trials: Participating in any clinical trials may be an option for some individuals, especially for advanced ACC cases. Clinical trials are research studies that help to test new treatment options.

Adrenocortical Carcinoma vs Pheochromocytoma

Adrenocortical carcinoma is a rare adrenal cortex cancer that tends to be associated with overproduction of hormones and rapid tumor growth, whereas pheochromocytoma is typically an adrenal medulla tumor that is benign in nature but results in elevated levels of adrenaline and sudden blood pressure spikes.

Living with Adrenocortical Carcinoma

Living with ACC can make you feel like a constant battle, especially since recurrence is common. It’s completely normal to experience anxiety, fear, and loneliness. But you are not alone, and there are many powerful strategies to help you cope.

Adrenocortical Carcinoma Life Expectancy:

Adrenocortical carcinoma is a cancer that recurs frequently, and this may create fear, loneliness, or anxiety. There are strategies for coping with these feelings, though. Connecting with support groups through your healthcare team can be incredibly comforting.

Programs focused on survivorship can also boost your overall well-being, particularly if the thought of the cancer relapse leads to anxiety. Such programs offer practical skills and emotional support to help you in this. If the patient is undergoing chemotherapy after surgery, palliative care can be an absolute game-changer, in managing side effects and maintaining your overall health. Remember, we're here to support you every step of the way.

Conclusion

Adrenocortical carcinoma, or ACC, is a hard and uncommon cancer, but with advancements in medical science and treatment, hope is present. Early detection and regular treatment can go a long way and enable many patients to control the disease and have a quality of life. In the case of children, ACC is particularly uncommon and aggressive, and therefore, it is vital to identify it at an early stage and obtain specialized treatment. A team-based model with specialists who know ACC offers the best chance at successful treatment and stable health.

Key Takeaway Note from iCliniq

Curious to know about rare adrenal cancers like adrenocortical carcinoma? You're in the right place for information. It is a rare cancer that often presents uniquely in diagnostic and treatment challenges. It can either overproduce hormones, causing different symptoms, or increase silently. Usually, surgery is the initial treatment, and sometimes it is followed by chemotherapy, hormone replacement, or radiation. We know living with ACC can surely feel overwhelming, but connecting with support groups, engaging in survivorship programs, and getting specialized care can definitely transform the experience. If you or your loved one is diagnosed with ACC, remember that our dedicated team of specialists at iCliniq is ready to help you, and personalized support and expert advice tailored just for you.