Adrenocortical Carcinoma is a rare disease characterized by the presence of malignant (cancerous) cells in the outer layer (adrenal cortex) of the adrenal gland. The adrenal gland is a small paired gland located above the kidneys and is also known as the adrenal cortex. The adrenal gland is composed of two layers: the outer layer (adrenal cortex) and the inner layer (adrenal medulla) in the center. The adrenal cortex is responsible for synthesizing important hormones.

These hormones have various functions, including regulating water and salt balance in the body, maintaining normal blood pressure, controlling the usage of proteins, fats, and carbohydrates in the body, and contributing to the development of male or female characteristics.

Adrenocortical Carcinoma, which is the malignant tumor of the adrenal cortex, can be either functional or non-functional. Functional tumors produce hormones, and most Adrenocortical Carcinomas are functional tumors that produce hormones. Excessive production of hormones by these functional cells can cause certain symptoms and signs of the disease.

The adrenal medulla produces hormones that help the body respond to stress. Cancer affecting the adrenal medulla is referred to as pheochromocytoma.

Adrenocortical Carcinoma and Pheochromocytoma can occur in both adults and children. The treatment approach for children is different from that of adults.

Genetic factors that increase the risk of developing Adrenocortical Carcinoma:

– Li-Fraumeni syndrome.
– Beckwith-Wiedemann syndrome.
– Carney complex.

A risk factor is anything that increases the chances of developing a disease. Having a risk factor does not mean that a person will definitely develop cancer, and not having any risk factors does not guarantee that a person won’t develop cancer. If you suspect that you might be at risk, it’s important to discuss it with your doctor.

Signs and symptoms of Adrenocortical Carcinoma:

– A lump in the abdomen.
– Abdominal or back pain.
– Feeling of fullness in the abdomen.

Non-functional tumors may not cause any symptoms.

For functional carcinomas, the excessive production of hormones can lead to the following symptoms:

Excessive cortisol production:

– Weight gain in the face, neck, and trunk, with thin arms and legs.
– Increased growth of fine hair on the face, upper back, or arms.
– Moon face: a round and red face.
– Fat deposits at the back of the neck.
– Deepening of voice and swelling of the sex organs or breasts in both men and women.
– Muscle weakness.
– High blood sugar.
– High blood pressure.

Excessive aldosterone production:

– High blood pressure.
– Frequent urination.
– Muscle weakness or cramps.
– Increased thirst.

Excessive testosterone production (in women):

– Increased hair growth on the face, upper back, or arms.
– Increased oiliness of the skin and acne.
– Balding.
– Deepening of the voice.
– Menstrual irregularities or absence of periods.

In men with excessive testosterone production, there are usually no symptoms or signs.

Excessive Estrogen Production (in Women):

– Irregular menstrual periods in premenopausal women
– Vaginal bleeding in postmenopausal women
– Weight gain

Excessive Estrogen Production (in Men):

– Enlargement of breast tissue
– Decreased libido
– Erectile dysfunction

These and other symptoms can also be caused by other conditions such as Adrenocortical Carcinoma. If you have any of these issues, it is recommended to consult your doctor.

How is Adrenocortical Carcinoma Detected and Diagnosed?

To detect and diagnose Adrenocortical Carcinoma, imaging studies and tests that examine blood and urine samples are used.

24-Hour Urine Test: This test involves collecting urine over a 24-hour period to measure the levels of cortisol or 17-ketosteroids. Elevated levels may indicate adrenal cortex pathology.

Low-Dose Dexamethasone Suppression Test: Cortisol levels are monitored in the blood or urine collected over three days after administering low doses of dexamethasone. This test is used to determine if the adrenal glands are producing excessive cortisol.

High-Dose Dexamethasone Suppression Test: Cortisol levels are monitored in the blood or urine collected over three days after administering high doses of dexamethasone. This test is used to check if the adrenal glands are producing excessive cortisol or if the pituitary gland is instructing the adrenal glands to do so.

Blood Chemistry Studies: These studies measure the levels of potassium or sodium released into the blood by organs and tissues. Abnormal levels may indicate an underlying condition.

CT Scan: A series of detailed images of different angles inside the body are taken using an X-ray machine connected to a computer. Contrast dye may be administered intravenously or ingested to enhance visibility of organs or tissues. This procedure is also known as computed tomography or computerized axial tomography.

MRI (Magnetic Resonance Imaging): This procedure uses a magnet, radio waves, and a computer to produce a series of detailed images of various areas of the body. It is also referred to as nuclear magnetic resonance imaging (NMRI). An abdominal MRI is performed to diagnose Adrenocortical Carcinoma.

Adrenal Angiography: It is used to examine the arteries near the adrenal glands and assess blood flow. Contrast dye is injected into the adrenal arteries, and a series of X-rays are taken to visualize any arterial blockages.

Adrenal venography: It involves examining the veins near the adrenal gland. Contrast dye is injected into the adrenal vein, and a series of X-rays are taken to see if any of the vessels are blocked. A catheter (thin tube) may be placed in the vein to obtain a blood sample for testing abnormal hormone levels.

PET Scan (Positron Emission Tomography scan): It is used to search for malignant tumor cells throughout the body. Radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and shows where glucose is being used. Malignant tumor cells appear brighter in the image because they are more active and utilize more glucose than normal cells.

MIBG scan: A small amount of radioactive substance called MIBG is injected into a vein and enters the bloodstream. Adrenal gland cells take up the radioactive material, which is detected by a radiation-measuring device. This scan is performed to differentiate between Adrenocortical Carcinoma and Pheochromocytoma.

Biopsy: It involves removing a suspicious cell or tissue from the area for examination of cancer characteristics by a pathologist. Tissues can be obtained using a fine needle aspiration (FNA) biopsy, which uses a thin needle, or a core biopsy, which uses a wider needle.

What are the factors that affect prognosis (chance of recovery) and treatment?

Prognosis (chance of recovery) and treatment options depend on the following:

– Stage of cancer (size of the tumor and whether it has spread to other parts of the body)
– Whether the tumor can be completely removed during surgery
– Previous treatment for cancer
– Overall health of the patient
– Grade of tumor cells (how different they appear from normal cells under a microscope)

Adrenocortical Carcinoma can be treatable if it is detected and treated in the early stages.

Spread of cancer in the body

Cancer can spread through tissue, lymph system, and blood. Through tissue, cancer spreads to nearby areas from where it started. When cancer spreads to another part of the body, it is called metastasis. Cancer cells separate from the primary tumor and travel through lymph system vessels or blood vessels to reach a different tissue, where they form a new tumor (metastatic tumor).

The metastatic tumor is the same type of cancer as the primary tumor. For example, if Adrenocortical Carcinoma spreads to the lungs, the cancer cells in the lungs are actually Adrenocortical Carcinoma cells. The disease is not lung cancer but rather Metastatic Adrenocortical Carcinoma.

Treatment approach for Adrenocortical Carcinoma:

Different treatment options are available for patients with Adrenocortical Carcinoma.

Three types of standard treatments are used:

– Surgery
– Radiation therapy
– Chemotherapy

Novel treatment approaches are being tested in clinical trials:

– Biological Therapy
– Targeted Therapy

Standard treatments:

1. Surgery

Adrenal gland surgery (adrenalectomy) is performed. Sometimes it may be necessary to remove the nearby lymph nodes and other tissues where the cancer has spread.

2. Radiation therapy

Radiation therapy is a treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or prevent their growth. There are two types of radiation therapy:

External radiation therapy uses a machine outside the body to deliver radiation to the tumor.

Internal radiation therapy involves placing a radiation source directly into or near the tumor. The radiation source is a radioactive material contained in needles, seeds, wires, or catheters.

The delivery of radiation therapy depends on the type and stage of the cancer being treated. External radiation therapy is used to treat Adrenocortical Carcinoma.

3. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to kill cells or stop them from dividing, thereby stopping the tumor’s growth. When chemotherapy is taken orally or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is applied directly to the cerebrospinal fluid, an organ, or a body cavity like the abdomen, the drugs affect the cancer cells in those areas (regional chemotherapy). Combination chemotherapy is a treatment using multiple anticancer drugs. The administration of chemotherapy depends on the type and stage of the cancer being treated.

Follow-up Tests

Some tests are repeated to diagnose cancer or determine the stage of the cancer. Certain tests should be repeated to assess how well the treatment is working. Decisions regarding the continuation, modification, or cessation of treatment are based on the results of these tests.

Some tests are periodically conducted even after treatment has ended. The results of these tests indicate whether there have been any changes in your condition or if the cancer has recurred. These tests are referred to as follow-up tests or check-ups.