Adrenal cancer: signs and symptoms, treatment, prognosis

About adrenal cancer

Adrenal cancer Is a rare condition that occurs in the adrenal glands, two tiny glands above the kidneys that are part of the endocrine system. Adrenal glands produce hormones that are responsible for a number of bodily functions, such as the body's ability to deal with stress and regulate blood pressure. Most tumors (carcinomas) of the adrenal glands are benign, which means they are not cancerous, and are called adenomas.

There are slightly more women among patients than men - about 60%. The peak incidence occurs at the age of 40-50 years.

Risk factors

Risk factors for adrenal cancer can vary. Like many types of malignant tumors, the risk of developing an adrenal tumor may increase in as a result of certain lifestyle and environmental factors, including smoking and exposure to carcinogenic substances. Genetic syndromes are also associated with adrenal cancer.

A rare form of adrenal cancer called pheochromocytoma occurs mainly in middle-aged adults. Although genetic syndromes and hereditary factors are associated with the occurrence of cancer cells, most cases of carcinomas occur sporadically, and disease triggers remain largely unknown.

Some genetic syndromes considered to be risk factors for the development of the disease include:

• Li-Fraumeni syndrome;
• von Hippel-Lindau disease;
• multiple endocrine neoplasia types 1 and 2 (MEN1, MEN);
• Beckwith-Wiedemann syndrome.

Adrenal cancer symptoms

Many of the symptoms of adrenal cancer, which are small glands located above each kidney, are associated with hormonal imbalance as a result of tumors that exceed or do not provide the secretion of a particular hormone. In malignant cancer (a tumor that can spread), symptoms often go unnoticed until the cancer is advanced.

The symptoms that develop vary depending on the stage of the tumor and the type of hormone secreted. The warning signs are often not obvious or seem unrelated, making it difficult to diagnose early-stage adrenal cancer. While blood and urine tests can detect elevated hormone levels, most tumors adrenal glands are found on imaging tests for other conditions and states.

Common symptoms of adrenal cancer include:

• fever (high temperature);
• a noticeable lump in the abdomen;
• constant pain (pressure of the tumor on the organs);
• a feeling of fullness caused by a tumor pressing on the stomach;
• unexplained weight loss.

Endocrine conditions such as Connes syndrome and Cushing's syndrome, are due to excess secretion of a certain hormone and can lead to adrenal tumors, although these tumors are usually benign.

Types of adrenal cancer

Differentiating between benign and cancerous adrenal tumors under a microscope can be difficult. According to the American Cancer Society, sometimes the only way to confirm cancer is the adrenal gland - when it has spread (metastasized) to lymph nodes or other organs and fabrics. Non-malignant tumors (adenomas) do not spread outside the adrenal glands.

There are three common types of adrenal cancer:

• Adrenocortical cancer: Also called adrenal cortex carcinoma, it is the most common form of adrenal cancer. Carcinoma usually forms in the outer layer of the cortex and is usually not found until the tumor is large enough. This type of cancer is often found after the onset of symptoms, especially pain or a feeling of fullness in the stomach, leading to weight loss. Adrenocortical carcinomas can also produce excess hormones that cause weight gain, excess facial hair (hirsutism), or early puberty. If an adrenal tumor is larger than 5-6 centimeters, it is usually considered malignant.
• Pheochromocytoma: This type of adrenal carcinoma forms in the central part of the medulla and usually originates from cells that produce adrenaline. Epinephrine helps regulate important bodily functions including heart rate and blood pressure. High blood pressure (arterial hypertension), increased sweating, heart palpitations (tachycardia) and anxiety are some of the symptoms of this type of tumor.
• Neuroblastoma: this type of carcinoma occurs in the developing nerve cells of the medulla. Neuroblastoma usually affects infants or children under 10 years of age. Due to the unique nature of the cells, early detection is possible. However, in rare cases, the origin can be difficult to determine, as cells can spread rapidly. According to the American Cancer Society, approximately one in three neuroblastomas start in the adrenal glands.

Diagnostics

In addition to taking a comprehensive medical history and examination, doctors at cancer centers can use a variety of tests and procedures to diagnose and stage adrenal cancer. The research methods used depend on factors such as the size and location of the tumor.

Blood and urine tests are done to check for irregular levels of adrenal hormones. These tests can detect swelling before symptoms appear.

Other tests used to diagnose the disease include:

• Radiography. During this examination, an image is taken of the chest and the surrounding area - the lungs, heart, large arteries, ribs and diaphragm. Smaller structures such as blood vessels are also checked. A chest x-ray can show if it has spread lung cancer and if there are abnormalities in the heart.
• Laparoscopy. During this minimally invasive procedure, a long, thin instrument with an attached camera (laparoscope) is inserted into the patient's side. The camera transmits images from inside the body, allowing the doctor to view any formations in the adrenal glands and involved lymph nodes without the need for surgery. Laparoscopy also helps the doctor determine if the cancer can be removed surgically.
• Computed tomography (CT). CT scans are used to determine if surgery is an acceptable cancer treatment option. A scan, usually done in 10-15 minutes, is used to determine if cancer has developed in the adrenal glands or other parts of the body, such as liver.
• Ultrasound examination (ultrasound). An ultrasound is performed when a CT scan cannot be performed. An ultrasound allows the doctor to look at the adrenal glands, check for tumors and potential masses in the liver where the tumor may have spread.
• Positron Emission Tomography (PET / CT). This test can be helpful in determining whether a tumor may be benign or malignant, and whether the cancer has spread beyond the adrenal glands.
• Magnetic resonance imaging (MRI). MRI can help distinguish between normal and abnormal tissue. In the case of disease, MRI can provide greater soft tissue contrast than CT. This type of imaging helps doctors evaluate tumors to determine if they are benign or cancerous. MRI can also be used to check the brain. Adenomas in the pituitary gland, located at the base of the brain, and adrenal tumors can cause similar symptoms.
• Biopsy. During this procedure, a sample of tumor tissue is examined under a microscope. Biopsies are usually performed only for tumors outside the adrenal glands. Puncture biopsy of adrenal carcinoma can lead to the spread of tumor cells through the bloodstream or other body fluids. This is why blood, urine, and imaging tests are used to diagnose the disease. A biopsy may be done to determine if tumors outside the adrenal glands are associated with adrenal cancer or caused by another tumor or disease.

Stages of adrenal cancer

Staging adrenal cancer is an important component in developing a treatment plan. Having the exact stage or progression of the disease is essential.

• Stage I: the tumor is less than 2 inches (5 centimeters) and has not spread outside the adrenal glands.
• Stage II: the tumor is larger than 2 inches (5 centimeters) but has not yet spread beyond the glands.
• Stage III: the tumor has spread to nearby tissues or lymph nodes. Size is not a factor.
• Stage IV: the tumor can be of any size and has spread throughout the body.

Adrenal cancer treatment

Surgical removal of the tumor, radiation therapy and chemotherapy are used to treat adrenal cancer. although how each will be used and whether they can be used in combination depends on the series factors. The oncologist surgeon will recommend one of several surgical approaches, depending on the stage of the disease.

In some cases of advanced adrenal carcinoma, chemotherapy can be used in combination with surgery to kill cancer cells or prevent their growth, and distribution.

Radiation therapy is often given after surgery for patients with advanced medical conditions to shrink or destroy cancer cells that cannot be removed during surgery.

- Surgical removal of the tumor.

Surgery is performed to treat all stages of adrenal cancer.

A minimally invasive procedure known as laparoscopy is the use of a thin, lighted tube with camera at the end, inserted through small incisions - may be an option for treating small tumors. In cases where laparoscopy cannot be performed, the following surgical procedures may be used:

• Back surgery​: this procedure removes the tumor through an incision in the back, just above the kidneys. This is usually the approach used to treat small, benign adrenal tumors.
• Transabdominal surgery: during the procedure, an oncologist surgeon makes an incision through the abdominal cavity to examine nearby tissues and organs, while removing the tumor of the adrenal gland, glands and other cancerous tissues found during procedures.
• Thoracoabdominal surgery: large cancers may require a long incision from the chest to the abdomen. This procedure allows a wider view of the surrounding tissues and organs in order to assess the potential spread and growth of the tumor.

- Radiation therapy.

Radiation therapy may be prescribed after surgery for advanced adrenal cancer. Radiation therapy serves several purposes, including the treatment of tumors that cannot be removed by surgery. by reducing the risk of tumor recurrence and treating areas where cancer has metastasized (e.g. brain, liver, bones).

- Chemotherapy.

Chemotherapy can be used in combination with surgery to treat advanced adrenal carcinoma. Chemotherapy drugs are designed to kill cancer cells or interfere with their ability to grow and multiply. Because chemotherapy does not have significant benefits for adrenal cancer, it is more likely to only used when the disease has metastasized widely so that it can be removed surgically by.

Survival rates (prognosis)

Survival rates can give you an idea of ​​the percentage of patients with the same type and stage. the cancer is still alive for a certain period of time (usually 5 years) after it has been delivered diagnosis. Survival rates cannot tell how long you will live, but they can help you better understand how likely treatment is to be successful.

These numbers are based on patients diagnosed with adrenal cancer between 2009 and 2015.

Stage	5-year relative survival
localized (stage 1-2)	74%
regional (stage 3)	56%
metastatic (stage 4)	37%