Adrenal Cancer

Adrenal

The Adrenal glands are also called suprarenal gland as it is situated above the kidneys and are a part of the endocrine system. They are yellowish, triangular-shaped organs located on top of each kidney. Adrenal glands are small, approximately 2.5 inches long and 1 inch wide. They produce a number of vital hormones essential for survival.

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Adrenal gland consists of three major parts:

  • The Capsule: This is a protective layer of fat that surrounds each Adrenal gland.
  • The Cortex: The cortex encloses the medulla and comprises around 80% of the volume of Adrenal gland. It produces different types of hormones called steroids viz minerals, corticoids, glucocorticoids and gonadocorticoids.
  • The Medulla: It is the innermost part of the Adrenal gland contributing 20% of its volume. The Adrenal medulla produces the Adrenaline (epinephrine)  and norAdrenaline (norepinephrine) hormones.

Functions:

  • Adrenal glands produce two most important hormones:  cortisol and aldosterone.
  • Aldosterone helps control blood pressure by managing the balance of potassium and sodium in the body.
  • Cortisol helps to maintain stress and regulate metabolism, sugar levels, and blood pressure.
  • The Adrenal glands also produce Adrenaline and small amounts of sex hormones called androgens.
  • The medulla secretes stress hormones that generate the primitive stress response.

Adrenal Cancer

Adrenal Cancer is a rare tumor that originates in one or both of the Adrenal glands. Since these glands are located deep inside the body so it becomes difficult to spot early symptom of the disease.

When cells in the Adrenal cortex become Cancerous, they start malfunctioning, synthesizing hormones more than required by the body. This results in several ailments like high blood pressure, weakening of the bones, or diabetes. 

Types of Adrenal Cancer

Depending on the place of birth of these Cancerous cells, Adrenal Cancer is categorized as:

  • Adrenocortical carcinoma: Originating in the outer layer of the cortex, it is the most common type of Adrenal Cancer. These tumors are usually discovered at a later stage when they have grown larger and cause prominent symptoms like pain, weight alterations or peculiar hormonal changes.
  • Pheochromocytoma: This is a relatively rare tumor of the Adrenaline-producing cells and only about 10% of pheochromocytomas are malignant. This is a familial Cancer with genetic predeposition. Hence, regular check-up will help the family members in early detection of the disease. An increased Adrenaline level, high blood pressure, excess sweating, and anxiety may be alarming signals about this disease.
  • Neuroblastoma: This originates in the nerve cells of medulla, Adrenal Cancer usually affects infants or children below the age of 10.

 Adrenal Cancer Occurrence Rate in India

Adrenal adenoma, a benign tumor is a common disease of the Adrenal glands but Adrenal Cancer, which is malignant in nature is a rare form. It accounts for only 0.2% of deaths due to cancer.

adernal-anatomy

The Adrenal glands are the production center for regulatory hormones such as Adrenaline and cortisone. So, most of the symptoms are related to hormonal imbalances resulting from abnormal secretion of a particular hormone. However, sometimes there may not be any distinct symptom until the tumor has grown big and presses against other organs causing a hindrance in their functionality.

Symptoms caused by a large size of the tumor:

  • Pain in the stomach or back.
  • Feel pressure or fullness soon after you eat.
  • A lump.

Symptoms caused by high level of androgen or estrogen:

  • Rapid growth of facial and body hair.
  • Enlarged penis in young boys or the clitoris in girls.
  • Depression, anxiety, and loss of emotional control
  • Cognitive difficulties
  • Early periods or breast development in young girls.
  • Breast growth or impotence in men.
  • Loss of sexual desire.
  • Irregular periods and change in voice.

Symptoms caused by high level of aldosterone

  • Conn’s disease: It is caused by excessive production of aldosterone, a steroid hormone responsible for regulating electrolytes. In the case of Conn’s disease, aldosterone becomes more active and sodium levels in the blood may increase thus affecting the blood pressure. Other prominent symptoms of this condition are frequent urination and excessive thirst.

Symptoms caused by an excess of stress hormone cortisol:

  • Unexplained alterations in body weight.
  • Stretch marks.
  • Weaker bones and muscles that get bruised easily.
  • Mood swings and depression.
  • High blood pressure or high blood sugar.
  • Cushing’s syndrome develops when a functioning adrenocortical tumor produces excess cortisol. Also, called the stress response hormone, cortisol also plays a significant role in regulating blood pressure and metabolic function.

The symptoms may vary depending on the type of Adrenal Cancer and the size of the tumor. Often, the warning signs of Adrenal Cancer are not obvious or the symptoms seem unrelated, thus making its diagnosis difficult. Blood and urine tests may help detect elevated hormone levels. However, physicians more often find an Adrenal tumor during a scan for an unrelated condition.

Till date, the exact causes of Adrenal Cancer are not known to precision, though there are many assumptions about the same. Throughout these years, it has been noted that one may be at a higher risk if they have any of the following conditions:

  • Genetic Conditions:
    • Li-Fraumeni syndrome
    • Beckwith-Wiedemann syndrome
    • Carney complex
    • Multiple endocrine neoplasias
    • Familial adenomatous polyposis
    • Lynch syndrome
  • Lifestyle Conditions:
    • Smoking: Tobacco use may increase the risk of developing Adrenal Cancer.
    • High-fat diet
  • Other
    • Exposure to Cancer causing radiations

Since the root cause of Adrenal Cancer is not known so, it is difficult to coin down the preventive measures. However, leading a healthy life does eliminate some risk of developing any disease including Adrenal Cancer.

Stages of Cancer are assigned to indicate the spread and severity of the disease. Based on the stage of Cancer the treatment and prognosis are decided. Adrenal Cancer can be staged into:

  • Stage I (T1, N0, M0): The tumor is smaller than 2 inches and has not spread beyond the Adrenal gland.
  • Stage II(T2, N0, M0): The tumor is larger than 2 inches but has not yet spread.
  • Stage III The tumor has spread to nearby tissues or lymph nodes. Depending on the size it is further subdivided into:
  • T1, N1, M0: The tumor is 5 cm or smaller and has spread to the regional lymph nodes.
  • T2, N1, M0: The tumor is larger than 5 cm and has spread to the regional lymph nodes.
  • T3, N0, M0:  The tumor may be of any size and has spread beyond the Adrenal glands.
  • Stage IV: The tumor may be any size, and tumors have spread throughout the body.
  • T3, N1.M0: The tumor has spread beyond the Adrenal gland to regional lymph nodes but not to distant organs.
  • T4, N0, M0: The tumor has spread to nearby organs, but not to lymph nodes
  • T4, N1, M0: The tumor has spread to nearby organs but not beyond them.
  • Any T, any N, M1: The tumor may have spread to any part of the body.

The survival rate is an analysis that reveals the percentage of people in a treatment group still alive for a given period after diagnosis. Survival rates are important to determine the prognosis.

The survival rate of Adrenal Cancer is high if it is detected at an early stage before Cancer has metastasized and the statistics gradually decreases with each passing stage.

However, the survival rate for each individual may vary to some extent based on their overall health condition, and treatment routine followed.

Stage5 year Survival Rate
Localized65%
Regional44%
Distant7%

Adrenal glands are located deep within the body which makes it difficult to detect the growing tumors. However, if the tumor interferes with the regulation of hormones then some symptoms might be visible. There are multiple tests and examinations to ascertain the cause of these symptoms.

  • Physical exam and medical history. The patient’s health habits and medical problems are scrutinized thoroughly. The doctor checks for menstrual and sexual functions along with the familial history of the patient.
  • Blood and urine tests: These tests are done to check for the amount of synthesis of sex hormones or steroids, such as low levels of potassium or high levels of cortisol or estrogen.
  • Imaging tests: Imaging tests are suggested if an Adrenal tumor or Cancer is suspected. Imaging tests are very useful to view the interior of the body and see if there are any tumors or cancer cells. Some of the imaging tests are:
    • X-rays: An x-ray shows if cancer has spread.
    • Ultrasound: Ultrasound tests use sound waves with the help of a transducer to take pictures of parts of the body. This test can show if there is a tumor mass in the Adrenal gland.
    •  Computed tomography scan: The CT scan produces detailed cross-sectional images of the body. It shows the Adrenal glands clearly and often confirms the location of the Cancer. It can also help show whether cancer has spread
    • MRI: MRI scans use radio waves and strong magnets instead of x-rays. MRI scans give a very detailed image of parts of the body.
    • Positron Emission Tomography (PET) scan: A radioactive glucose is injected into the vein because Cancer cells use sugar much faster than normal tissues, radioactivity will tend to concentrate in the Cancer. A scanner can spot the radioactive deposits. This test can be helpful in spotting small collections of cancer cells and may be used to find cancer that has spread.
  • Laparoscopy: A very thin tube with a tiny video camera is inserted into your body. A laparoscopy test shows places where Cancer is growing.
  • Biopsy:  A tiny sample of tissue is extracted from the tumor with the help of a needle. The cells are then viewed under a microscope to see if there is any Cancerous development.

The treatment plan usually depends on the stage and the patient’s preferences.  Based on these factors a complete treatment plan is prepared and the treatment may consist of one of the following procedures:

Surgery: Surgery is the most preferred treatment option that is able to provide a complete cure. In surgery, one or both of the Adrenal glands may be removed. If the cancer cells have spread beyond the Adrenal glands then nearby lymph nodes are also taken out.

Radiation: Radiation is a treatment procedure can kill Cancer cells or inhibit the growth of the tumor. Radiation may be given internally by inserting radioactive seeds inside the body or externally through a machine. 

Chemotherapy: Chemotherapy is the use of drugs to control the growth of cancerous cells. For Adrenal Cancer, doctors most often prescribe a drug called mitotane (Lysodren), which blocks the Adrenal gland from making hormones. It also destroys cancer cells.

Some chemotherapy drugs that go through the whole body to kill Cancer cells, but they can also harm healthy cells, too.

Targeted therapy drugs look for and kill Cancer cells without harming good ones.

Tumor ablation. In this procedure, heat or cold is used to kill Cancer cells. This helps in relieving the symptoms and give a better quality of life.

Adrenal Cancer is highly curable when detected early. In some cases, an operation to remove the tumor cures the disease. There are several other procedures to keep the recurrence of Cancer in check.

In case of experiencing any of the mentioned symptoms, it is advisable to visit the doctor at the earliest. Also, a person having a high risk of developing Adrenal Cancer should go for a diagnosis once or twice a year.

Being aware of the symptoms, and any health issue that might indicate the presence of the disease needs to be examined thoroughly. Also, a few lab tests and regular tests can also sometimes unveil the presence of the hideous disease.

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