Adrenal Cancer and Adrenal Tumors – DiseaseFix Patient Manual

Adrenal cancer overview: What is adrenal cancer?

Adrenal cancer is a rare type of cancer which begins in the cells of adrenal glands. These glands are a part of endocrine system and they produce a vital number of hormones essential for survival.

What are Adrenal glands?

Adrenal glands are small triangular structures, placed one over each kidney. They are yellow in color and both weigh about 10grams in total. The adrenal glands measure approximately 3.0 cm in width, 5.0 cm in length, and up to 1.0 cm in thickness.

The function of adrenal gland is to release hormones in the blood system. Hormones are essential in maintaining many body processes, which include metabolism, sexual development and puberty, and stress, etc.

Parts of adrenal gland

Each adrenal gland has two parts:

Cortex

The outer part, cortex, is responsible for the production of certain hormones with similar chemical structures known as Steroids. They are of three types:

• Glucocorticoids, such as cortisol, regulate body metabolism affecting the way body uses fats and carbohydrates and also manage the stress levels.
• Mineralocorticoids, such as aldosterone, help the kidney to maintain sodium and water levels in the body. It also regulates the blood pressure and maintains the amount of blood in the heart and circulatory system.
• Sex hormones, such as adrenal androgens (converted to estrogen and testosterone in other parts of the body), are produced in small amounts. Male hormones are androgens produced by testicles and female hormones are progesterone and estrogen produced in ovaries. Although, some amounts of androgens and estrogens are produced in both sexes.

Medulla

Medulla is the inner part of the adrenal gland and mainly functions to produce hormones called as catecolamines. The adrenal medulla makes three different catecolamine hormones: epinephrine, norepinephrine, and dopamine which control the body’s responses to stress, including the “fight or flight” adrenaline surge. These “stress hormones” have the potential to increase alertness, strength, and speed in an emergency. They also affect our heart rate, blood pressure, slows down the digestion process, and cause sweating.

Medulla is supposed to be an extension of nervous system. Tumors and cancers that begin in the adrenal medulla can be pheochromocytomas and neuroblastomas. Pheochromocytomas are mostly benign.

Adrenal cancer usually occurs in the outermost layer of the glands, or the adrenal cortex and appears as a tumor.

Types of Adrenal tumors (adrenal cancers)

Tumors of the adrenal gland (adrenal mass) can develop in both the cortex and the medulla. Tumor begins when cells grow out of control resulting in the formation of an unhealthy mass of cells. An adrenal gland tumor is commonly of any of the four types:

Adrenal adenomas

Adenomas (or adrenal adenoma) are the most common type of adrenal gland tumors which appear on the cortex without any symptoms in the initial stage. This type of tumor is generally small in size and benign (non-cancerous or non-functioning) in nature. They often appear only on one adrenal gland and it is rare to find them on both glands. It is also known as Adrenal cortex adenoma.

Adenomas are also known as incidentalomas, as they have no specific symptoms and are found by accident when CT or MRI scan is done of the abdomen for any other condition’s diagnosis. Only 15% of adenomas are found to be functional i.e. hormone producing.

The treatment of adenomas depends on the production of the hormones resulting in the difference in tumor size. If the tumor remains small and there is no hormone production, the treatment might not be needed. The tumors are watched for some time (approximately for 6-24 months) through scans (CT and MRI). If they secrete hormones and continue to increase their size, they are supposed to be functional and malignant and they may be removed through surgery.

Adrenal cortical carcinomas (ACCA or adrenal carcinomas)

Adrenal cortical carcinomas orsimply adrenal carcinoma are much larger than adenomas. They are known as Adrenocortical carcinomas (ACC or carcinomas). Cortical means cells of the cortex. This type of cancer develops on the cortex of the adrenal gland and produces hormones which bring changes in the body. It is a rare cancer and a study suggests that it occurs in 1-2 people per million of the population every year.

ACC is supposed to be a highly aggressive cancer and therefore, is simply often called as adrenal cancer. Tumors of ACC are only found when they have become very large and have started causing adrenal cancer symptoms like pain (caused by pressing other organs in the abdomen), fluid retention resulting in feeling of fullness, hormone secretion which results in weight gain, growth of hair in women, puberty in children, etc.

Adrenal tumor larger than 5 centimeters (about 2.5inches) is supposed to be cancer. They are also removed by surgery.

Neuroblastoma

Neuroblastoma is sometimes referred to as childhood cancer that affects the nerve cells in brain, pelvis, or in adrenal medulla (adrenal medulla cells) of the body of infants and children. Neuroblasts are immature nerve cells found in unborn babies. Normally, these cells mature as adrenal medulla cells in adrenal medulla. Neuroblastoma occurs when these cells don’t mature properly but only grows forming a mass called tumor.

Neuroblastoma generally occurs in children with an average age of 5 and sometimes even before the birth of the child. It is the third most common type of cancer in children. It can be found, but rarely, during the ultrasound of the mother if its formation has started before the birth of the child. The approximate survival rate for this disease is 80%.

Pheochromocytoma

Pheochromocytoma is a type of neuroendocrine cancer that mostly begins in adrenal medulla. It is a rare and usually benign cancer. Pheochromocytoma causes adrenal glands to make too many stress hormones called epinephrines and norepinephrines. At times, this type of cancer occurs outside the adrenal gland (somewhere in the abdomen) and is therefore known as extra-pheochromocytoma or paragangliomas. But, usually it occurs in the centre of the medulla from adrenaline (that regulates body functions such as heartbeat, blood pressure) producing cells.

Statistical analysis for adrenal cancer: Survival rate, prognosis, outlook

Adrenal cancers are very rare and are majorly found in people of middle age and the average age of a person diagnosed with adrenal cancer is around 45. Women are more prone to such tumors.

What is the 5-year survival rate of adrenal cancer?

Experts compute the survival statistics every 5 years. The 5-year survival rate for people with adrenal cancer is based on several different factors, such as the extent of cancer when diagnosed, the family history of the person, etc. Other factors that affect the survival statistics are age of the patient, whether the tumor produces hormones, etc. The 5-year survival rate explains the percent of people living at least 5 years after the cancer is found.

The 5-year survival rate is 65% if the cancer is detected inside the adrenal gland. If it has spread to the nearby tissues, lymph nodes and organs, the survival rate is 44%. The survival rate is minimum i.e. 7% if the cancer has spread to any distant part of the body.

Risk factors of adrenal cancer: Am I at an increased risk for adrenal cancer?

Risk factors explain the chances of developing a disease. Different diseases (cancers specifically) have different risk factors. A risk factor can be a behavior, substance or a condition.

Certain risk factors are more likely to cause adrenal cancer but none of them are completely understood. Scientists are researching to understand better about these risks factors, their mechanisms, and why some people develop adrenal cancers but others do not.

Genetic factors that affect adrenal cancer: Is adrenal cancer genetic?

The first and foremost factor leading to adrenal cancer is hereditary disorder (genetics of a person). Although, only 15% of the people suffer with adrenal cancer caused by genetic disorder, the maximum of them are children.

A few genetic syndromes that are considered as likely risk factors for adrenal cancer are discussed below:

Li- Fraumeni syndrome

Li-Fraumeni syndrome is a genetic disorder resulting in the mutation of a gene called TP53, which causes its de-functioning. Other cancers associated with this syndrome are bone cancer, breast cancer, brain tumors, soft bone sarcomas, etc.

Beckwith-Wiedemann syndrome(BWK)

BWK affects the growth of different body parts i.e. it marks abnormal growth disorder especially for large organs. Liver, kidney, adrenal cortex have an increased risk of adrenal cancer due to this syndrome.

Von Hippel-Lindau disease

In this condition, tumors arise from different organs. The cells lose their ability, due to mutation, to process information properly and act as if they are starved of oxygen. The abnormal cells affect the normal cells around them and produce abnormally high numbers of blood vessels resulting in the formation of tumors and cysts in those areas.

Adrenal glands, eyes, pancreas, spine, some parts of brain, etc are majorly affected by Von Hippel-Lindae syndrome.

Multiple endocrine neoplasia (MEN type1 and MEN type2)

Multiple Endocrine Neoplasia affects the endocrine system resulting in the development of tumors in three glands:

• the pituitary
• parathyroid
• pancreas

MEN1, known as Wermer’s syndrome, is caused by mutation in MEN1 gene which disables tumor suppression mechanism which leads to division of cells, thus resulting in formation of tumor. MEN2 causes mutation in RET gene which increases the risk (to 95%) of forming medullary thyroid tumor.

Other related problems can also occur such as adrenal tumors (pheochromocytomas), curvature of spine, thickening of eyelids, etc.

Some other genetic syndromes for adrenal cancer

There are a few other syndromes that can increase the risk of adrenal cancer. Example are – Familial adenomatous polyposis (FAP), Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC), Neurofibromatosis type 1 (also known as von Recklinghausen’s disease), Paraganglioma syndrome, Gardner syndrome, Carney triad, Cowden syndrome, Turcot syndrome, etc.

Age

Age is another risk factor for adrenal cancer. Certain types of neuroendocrine tumors affect people of certain ages, for example:

• Carcinoid tumors are often diagnosed in a person in his or her 50s and 60s.
• Neuroblastoma occurs only in children at about 5 years of age.
• Pheochromocytoma usually starts in middle-aged people, at around 40 to 60.
• Merkel cell cancer tends to happen at above 70 years of age usually.

Adrenal tumors can be considered to be age dependent.

Lifestyle and environmental factors

Lifestyle and environmental factors play some role in the development and progression of adrenal cancer. Smoking, exposure to cancer causing substances, pollution, being overweight, etc are some factors that can lead to the formation of tumors in the adrenal gland.

Do we know about the causes of adrenal cancer?  What causes adrenal cancer?

The exact causes for tumor development are still unknown. Over the years, a few studies have predicted that certain mutations in a person’s DNA can cause the formation of cancerous cells in the adrenal gland. The mutations can be due to different reasons. It may be due to heredity factors or other physiological factors.

Doctors refer the adrenal risk factors as the basis of formation of tumors leading to cancer in the adrenal glands. These factors are related to the genes and metabolism of a person. The heredity or mutations caused in the genes are the most important factors triggering the causes of adrenal cancer.

Gender often has a role to play during the development of adrenal cancer in a person. Certain studies and statistics have shown that men suffer more with pheochromocytoma and Merkel cell cancers and women is at a slightly higher risk for carcinoid tumors.

The exposure to radioactive radiations, effects of change in environment, living habits such as smoking or alcohol consumption, are some external factors which might initiate the beginning of tumors in the adrenal gland.

Prevention of adrenal cancer: Can adrenal cancer be prevented?

Adrenal cancer is a rare condition. The root cause of the disease is still unknown and therefore there is no preventive measure known as yet. The only thing a person can do to reduce the risk of not only adrenal cancer but all types of cancers is to maintain a healthy lifestyle and early screening in people who are at increased risk of the cancer.

Signs and Symptoms of Adrenal Cancers

There are no early signs and symptoms of Adrenal cancer. Adrenal cancer symptoms and signs become prominent when the tumor grows in size and the hormonal imbalance causes changes in the body.

At times, the cause of these symptoms may be some other medical condition that is not a tumor. Approximately, about 60% of patients are detected with symptoms due to high levels of hormones in the blood.

General signs and symptoms of adrenal cancer include:

• Fever
• Feeling of “fullness” (caused by a tumor or adrenal nodule pressing against the stomach)
• Persistent pain (Abdominal pain-pressure of tumor on organs)
• Heart palpitations
• Nervousness
• A noticeable lump in the abdomen
• Unexplained weight loss or gain
• Excessive hair growth
• High blood pressure
• Diabetes

The symptoms of adrenal cancers often depend on the type of tumor or adrenal nodule being formed:

Functioning tumors

These tumors may cause symptoms because of the excess amounts of particular hormones produced. The symptoms depend on the hormone that is being overproduced.

Non-Functioning tumors

Non-Functioning tumors do not produce high levels of hormones. The symptoms arise after a long period of time due to the position or size of the tumor mass. Increase in the size of the tumor builds pressure on the surrounding organs which may cause symptoms such as pain, bloating, etc.

Symptoms of adrenal cancer caused by different hormones

The symptoms of adrenal cancer caused by an increase in hormone production are categorized based on different hormones , such as:

• Cortisol
• Androgens or Estrogens
• Aldosterone
• Epinephrine or Norepinephrine

Symptoms of adrenal cancer caused by excessive Cortisol production

The main problem that is caused by excess level of cortisol is Cushing syndrome. Cortisol, produced in the adrenal cortex (outer part of the adrenal gland), is an important hormone that regulates blood pressure and other important metabolic functions of the body. It is also known as “stress hormone”.

What is Cushing’s syndrome?

A functioning adrenocortical tumor produces excess cortisol which leads to Cushing syndrome. Although, Cushing’s syndrome is also caused by pituitary gland and excess use of drugs during different treatments, it mainly occurs due to a functional adrenal tumor. Benign pituitary gland tumors can produce high levels of another hormone called adrenocorticotropic hormone (ACTH).

The signs and symptoms of Cushing syndrome include:

• Weight gain around the chest (neck or collar bone) and stomach(abdomen)
• A red, bloated face (moon face)
• Weakening of muscles and fatigue
• Diabetes and high blood pressure
• Excessive hair growth on the face and body in women
• Minor skin disorders such as acne or severe dryness, the appearance of purple stretch marks on the abdomen, thighs, breasts or arms, etc.
• Depression and/or moodiness(mood swings)
• Weakening of bones (Osteoporosis) which can sometimes cause fractures.
• Menstrual irregularities known as Amenorrhea

Symptoms caused by excessive androgen or estrogen

Large amount of androgen and estrogen secretion can cause adrenogenital syndrome. Symptoms of excessive production of androgen or estrogen can easily be spotted in children than adults because signs such as puberty and other physical changes are more active and visible in them. Diagnosing a tumor is way more difficult for women with excess estrogen and men with excess androgen.

Some signs and symptoms of adrenal cancer in children can be:

• excessive pubic, underarm, and facial hair growth
• early puberty in girls
• large breasts in boys
• an enlarged penis
• an enlarged clitoris

Symptoms of excessive androgen production in women:

• Excessive hair growth on the face, arms or upper back or sometimes baldness (a receding hairline)
• Acne
• No menstrual periods
• Deepening of the voice

Symptoms of high estrogen production in women:

• Irregular menstrual periods in premenopausal women
• Menstrual bleeding in post-menopausal women

Symptoms of too much estrogen production in men may cause:

• Breast enlargement
• Decreased sex drive
• Impotency (erectile dysfunction)

There are no usual symptoms for excessive androgen production in men.

Symptoms caused by aldosterone production

Adrenal gland tumors that produce high amount of aldosterone (a mineralocorticoid, produced in adrenal cortex) in the body can cause Conn’s syndrome (primary hyperaldosteronism).

What is Conn’s disease (or Conn’s syndrome)?

Aldosterone regulates electrolytes. In Conn’s disease, aldosterone becomes more active leading to an increase in sodium levels in the blood. This affects the blood pressure causing hypertension. Extreme high blood pressure could be life threatening.

The signs and symptoms of Conn’s syndrome include:

• High blood pressure
• Weakness or fatigue
• Low blood potassium levels
• Muscle cramps
• Heart problems
• Increased thirst
• Frequent urination
• Frontal headaches
• Changes in vision
• Abnormal sensation on the skin (paraesthesia), such as numbness, prickling, tingling or burning.

Symptoms caused by too much epinephrine or norepinephrine

Functional pheochromocytomas can make excessive amounts of epinephrine or norepinephrine. High levels of these hormones can cause health problems.

Signs and symptoms may include:

• High blood pressure
• Headache
• Sweating
• Rapid heart beat

Early detection and screening for adrenal cancer: Can adrenal cancer be detected at an early stage?

An early diagnosis of adrenal gland cancer is difficult. These cancers are very slow growing and often cause no symptoms during the early stage of cancer development. Adrenal tumors are generally detected when they get very large or spread to other nearby regions. Mostly, they are accidently found through scans of the abdomen for any other health issue or in a routine exam.

The adrenal glands secrete hormones and functions as the neuro-endocrine system of the body. But, sometimes, hormone imbalances in the body can promote early signs of tumor development in the adrenal glands of the body, which can help in early detection of the symptoms of adrenal cancer too.

Tests and Diagnosis of adrenal gland cancer: How do you test for adrenal cancer?

Diagnosis is the process of finding out the cause of a health problem and then analyzing the severity of the condition. The appearance of symptoms is the best way to detect adrenal gland cancer, but these could be due to some other conditions as well such as adrenal cysts etc. Therefore, doctors order certain tests to rule out other possibilities and make a confirmed diagnosis of the adrenal gland cancer.

Tools, procedures, and tests for adrenal cancer diagnosis

Several diagnostic methods are used for the detection of adrenal cancer:

Health history and physical exam

• Health History: The health of a person and the medical history can suggest about the beginning of adrenal gland cancer. The hereditary (family history) is also linked to an increased risk of adrenal gland cancer.
• Physical exam: A physical exam allows the doctor to look for certain signs of adrenal gland cancer. During a physical exam, the doctors check the blood pressure and feel the abdomen for enlarged organs or a lump.

Blood and urine tests for adrenal hormones to detect adrenal cancer

Blood and urine tests measure the levels of adrenal gland hormones and the products of their breakdown (metabolites) in the samples. Often doctors verify hormone levels even when symptoms of high hormone levels are not present because the symptoms of abnormal hormone levels can be mild. Blood tests might be able to detect these changes in the hormone levels even before the symptoms appear.

Tests for high cortisol levels

A high cortisol level predicts the presence of an adrenal gland tumor. Dexamethasone is a drug that is given to patients before examination. It acts like cortisol. If the person is suffering with adrenal tumor, the hormone levels remain high in the blood after they receive dexamethasone or else the hormone levels will become lower than normal. The test is called Dexamethasone suppression test.

Blood levels of another hormone called ACTH are also measured to help distinguish adrenal tumors from other diseases that can cause high cortisol levels.

A 24-hour urinary metanephrine and normetanephrine level test also helps to measure the hormones called catecholamines produced by the adrenal medulla and to verify for a pheochromocytoma. Uplifted levels of epinephrine or norepinephrine predict the presence of pheochromocytoma.

Tests for high androgen or estrogen levels and aldosterone tests

• Patients with estrogen-producing tumors will have high levels of estrogen in their blood.
• High levels of aldosterone mean that there is an adrenal gland tumor making aldosterone. This can also lead to low levels of potassium and renin (a hormone made by the kidneys) in the blood.
• Increased level of follicle-stimulating hormone (FSH) means that there is probably no adrenal gland tumor producing too much androgen or estrogen.
• Patients with androgen-producing tumors will have high levels of dehydroepiandrosterone sulfate (DHEAS) or testosterone.

Imaging tests for adrenal gland cancer diagnosis

Chest x-ray

Small doses of radiations (x-rays) help in the formation of images of body parts on a film which can detect the spread of cancer to the lungs. It is also useful in finding serious lung diseases or heart diseases too.

Ultrasound

Ultrasound tests use high frequency sound waves to produce images of parts of the body. The sound waves are reflected off the tissues and organs in the body with the help of a device called transducer. The pattern of sound wave echoes are then detected and analyzed by a computer to create an image of tissues and organs with cancerous locations.

The test is not usually used for the diagnosis of adrenal cancer. Instead, it is recommended to find out adrenal tumor. It can also confirm tumors in the liver if the cancer has spread there. Generally, ultrasound is not used for adrenal tumors unless a CT scan can’t be done for some reason.

Computed tomography (CT)

A computed tomography (CT) scan uses special x-ray equipment to make 3-D and cross-sectional images of organs, tissues, bones and blood vessels inside the body. A computer turns the images into detailed pictures. A CT Scan can clearly show an adrenal tumor with confirmed location. It also depicts the spread of the cancer to nearby organs and shows lymph nodes. It also helps to find out if a tumor is cancerous or non-cancerous based on its features, such as the size, the density and whether the edges are smooth or uneven.

Magnetic resonance imaging (MRI)

Magnetic resonance imaging (MRI) mechanizes powerful magnetic forces and radiofrequency waves to make cross-sectional images of organs, tissues, bones and blood vessels instead of using X-rays.

MRI provides more information than CT scans as it can better distinguish adrenal cancers from benign tumors.

MRI scans are very helpful in examining the brain and spinal cord. Tumors of the pituitary gland, which lies underneath the front side of the brain, can cause symptoms and signs similar to adrenal tumor.

MRI also helps to distinguish between a cancerous or non-cancerous tumor on the basis of its features, such as the size, the density or whether the edges are smooth or uneven.

Positron emission tomography (PET)

A positron emission tomography (PET) scan uses radioactive materials called radiopharmaceuticals in the form of sugar which are injected into the body. The cancer cells collect due to the effect of these radioactive materials. There are changes in the metabolic activity of body tissues. A special computer analyzes the radioactive patterns in the body and makes 3-D color images of the area being scanned.

PET scan is of great help in deciding if an adrenal tumor is benign or malignant (cancer), and if it may have spread to other parts of the body.

Other scans and tests for adrenal cancer

• Laparoscopy for adrenal cancer: Laparoscope is a thin, flexible tube with a very tiny video camera on the end. The growth of cancer is studied by the surgeons when this device is inserted through a small surgical opening in the patient’s side. It helps in spotting distant spread as well as enlarged lymph nodes (which might be cancerous). The benign adrenal cancers are at times removed also through this method. Laparoscopy is also done to predict the possibility of completely removing a cancer via surgery.
• Biopsy for adrenal cancer: The tissues or cells (tumor mass) are taken from the suspected site for testing in a laboratory. This is called Biopsy of the sample. Radiologists perform biopsy and pathologists analyze the sample to detect the presence of cancer cells.Fine needle aspiration (FNA) or biopsy uses a very thin needle to remove a small amount of fluid or cells from a lump or mass. Core biopsy uses a probe or hollow needle to remove a piece of tissue so it can be examined under a microscope. A biopsy of an adrenal gland can sometimes cause serious problems such as collapsed lung (pneumothorax), excessive blood loss (hemorrhage), and sudden increases in hormone levels (catecholamines).
• Other scans and tests for adrenal cancer diagnosis: Bone scan, Adrenal vein sampling, Metaiodobenzylguanidine (MIBG) scan, etc are a few scans and tests which help to detect and diagnose the adrenal cancer.

What are the various stages of adrenal cancer?

In general, patients with adrenocortical cancer are divided into 4 groups or stages of adrenal cancer based on the AJCC TNM staging system.

The standard AJCC TNM staging system and staging of adrenal cancer

Tumor (T)

The letter “T” along with a letter or number (0 to 4) describe the size and location of the adrenal tumor.

TX: The primary tumor cannot be evaluated.

T0: There is no primary tumor.

T1: The tumor does not grow outside the adrenal gland and the size is 5 centimeters (cm) or less.

T2: The tumor here also doesn’t grow outside but is larger than 5 cm.

T3: The tumor has grown into the area around the adrenal gland and can be of any size but has still not spread to nearby organs.

T4: The tumor is of any size. It has grown into any of the following:

• To nearby tissues or organs, such as the kidney
• Diaphragm: This is the thin muscle under the lungs and heart that separate the chest from the abdomen
• In larger blood vessels, such as the aorta and the vena cava
• Pancreas, spleen, liver.

Node (N)

The letter “N” in the TNM staging system stands for lymph nodes. Lymph nodes are tiny, bean-shaped organs that help fight infection.

Lymph nodes near the area where the tumor started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

NX: The regional lymph nodes cannot be evaluated.

N0 (N plus zero): The cancer has not spread to the regional lymph nodes.

N1: The cancer has extended to the regional lymph nodes.

Metastasis (M)

The letter “M” in the TNM system indicates whether the cancer has extended to other parts of the body, called distant metastasis (metastatic cancer).

M0 (M plus zero): The cancer has not stretched to other parts of the body.

M1: The cancer has spread to other parts of the body further than the nearby organs.

The 4 stages of adrenal cancer are:

• Stage 1: The cancer has not spread outside the adrenal gland and is about or smaller than 5 centimeters (2 inches) (T1, N0, M0).
• Stage 2: The tumor is larger than 5 cm, but has not grown outside the adrenal gland (T2, N0, M0).
• Stage 3: The cancer has grown into nearby tissues or lymph nodes. It spreads to the lymph nodes or other organs such as kidney, liver, etc near the adrenal gland (T1/T2/T3/T4,N1, Any N, M0)
• Stage 4: The cancer can be of any size and has spread to adjacent organs / other parts of the body such as to the colon, bone or lungs and is called distant metastasis cancer (Any T, Any N, and M1).

Treatments for Adrenal Cancer: Can adrenal cancer be treated or cured?

Early detection and treatment has the maximum possibility for curing the adrenal cancer. The treatment methods of adrenal cancer depend on several factors such as:

• The stage of the adrenal cancer
• Type of tumor (functional or non functional)
• Whether or not the tumor can be removed by surgery
• The general health of the patient

How is adrenal cancer treated?

There are several ways to treat the adrenal gland cancer.

Surgery for adrenal gland cancer: Can you cure adrenal cancer with surgery?

Various adrenal gland cancer surgeries are available for cancer treatment. Your doctor will decide which suits you the most based on your condition, the stage and spread of the cancer.

Adrenalectomy

Surgery is supposed to be the main treatment method which involves the removal of adrenal gland completely. This method is known as adrenalectomy. Along with the main gland, the nearby affected parts where the cancer has spread are also removed. If the lymph nodes near the cancer are enlarged, they may also need to be removed and checked for cancer spread. If the tumor is causing a blockage, for example, in the bowel, surgery may be helpful to relieve the blockage.

En bloc resection

Another type of surgery is En bloc resection that removes the tumor and surrounding tissues as a single piece. It is mostly done for adrenocortical carcinoma (ACC) that has spread to nearby tissues.

Laparoscopy

Sometimes, small adrenal tumors are removed through Laparoscopy (already discussed above). The main advantage of this method is that patients can recover from the surgery more quickly due to small incisions.

The disadvantage of this method is that it cannot be used for larger tumors. The tumors when broken down into small pieces increase the risk of the cancer to spread to different nearby organs.

Surgery for adrenal cancer metastasis (metastatic adrenal cancer)

This kind of surgery is done to remove the adrenal gland cancer that has spread (metastasized) to other organs such as the liver, kidney, spleen, pancreas and lymph nodes. Surgery is also the main treatment method for a pheochromocytoma, the tumor of the adrenal medulla that produces excess catecholamines.

Side effects of surgical methods

• Adrenal gland surgery can cause bleeding
• Excessive stress hormone are produced leading to high blood pressure
• Surgery for adrenal gland cancer may cause pain, decreased urinary output, changes in blood pressure and low blood sugar.

Radiation Therapy for Adrenal Cancer

The radiation therapy uses high beam of radiations that destroy the adrenal cancer cells. Radiation therapy may involve targeted energy (for example, X-rays, radioactive substances) to destroy cancer cells or shrink the tumors.

Sometimes, radiation therapy is used after the surgery to keep the tumor from coming back. This is called adjuvant therapy. Adrenal tumors need radiation therapy in rare cases only. The radiations are used:

• when the tumors cannot be removed during surgery
• to reduce the risk of tumor recurrence
• to treat areas such as bone, spine, brain where cancer has metastasized

Types of radiation therapy

External beam radiation therapy

External beam radiation therapy is generally done after ACC surgery to minimize the risk of cancer returning back. This radiation therapy also works when the cancer starts spreading to the brain or in bones.

The radiations are directed from outside the body to the cancerous tumor cells inside the body. It is not a painful method and doesn’t take a long time to complete the procedure. The radiation angles are accurately measured and proper dose of radiations are aimed correctly for the best results.

Brachytherapy (internal radiation therapy)

Small pellets of radioactive material are used to cure the adrenal cancer. The pellets are directly placed on the adrenal tumor mass or in thin plastic tubes. These tubes are also placed on the tumor for a few days and then removed. A reduction is observed in the adrenal cancer mass. Adrenal cancer requires high dose brachytherapy. Adrenal cortical cancer cannot be treated with this therapy.

Other Radiation Therapy for adrenal cancer

Targeted radiation therapy with radioactive metaiodobenzylguanidine (MIBG) treats cancerous pheochromocytomas. The radioactive material is delivered directly into the cancer cells when MIBG attaches to the adrenal cancer cells. This kind of radiation therapy is rarely used and may not be available at all treatment clinics.

Common side effects of radiation therapy for adrenal cancer:

Radiation therapy mainly depends on the size of the tumor or the size of the organs to be treated or the size of the area affected by cancer. The dose and schedule of the radiation therapy is dependent on many other factors also. Radiations have some side effects as mentioned below:

• Fatigue, nausea and vomiting
• Loss of appetite
• Diarrhea (if an area of the abdomen is treated)
• Skin problems in the area being treated, which can range from redness to blistering and peeling
• Loss of hair in the area being treated
• Low blood counts

Chemotherapy for Adrenal Cancer

Chemotherapy refers to a combination of medications (certain cytotoxic drugs) used to kill the cancer tumor cells. Depending upon the stage of cancer, the doctor prescribes a combination of surgery and chemotherapy to treat advanced stages of the cancer.

Usually, if the tumor is removed once, the chances of recurrent growth of the adrenal cancer cells are very less. A chemotherapy schedule is divided into certain number of periods and cycles which depend on the time required to shrink the tumor and the size of the tumor.

In adrenal cancer, chemotherapy is mainly done at an advanced stage after surgery because it does not work well in the initial stage of the adrenal cancer. It is often used when the adrenal cancer has metastasized (spread) to other organs widely. It is also used to minimize the symptoms caused by excessive production of certain hormones in advanced adrenal gland cancer.

Chemo drugs are given either intravenously (injected through veins or muscles) or orally (through mouth as pills). These anticancer drugs reach throughout the body by entering into the bloodstream and treating the cancer that has spread in various parts of the body beyond the adrenal gland.

Drugs used for adrenal cancer chemotherapy

Mitotane: Mitotane, also known as Lysodren, blocks the hormone production in adrenal gland. This kills the tumor or cancer cells. It is used to treat the cancer of adrenal cortex by reducing the amount of production of adrenocorticoids in the adrenal cortex. The disadvantage is that it kills the healthy cells of the body also.

It often leads to a decrease in cortisol production causing weakness and fatigue. It also reduces the production of testosterone or thyroid hormone.

Other common drugs used for chemotherapy of adrenal gland:

• Cisplatin
• Streptozocin plus mitotane
• A combination of cisplatin, doxorubicin (Adriamycin), and etoposide (VP-16) plus mitotane
• Doxorubicin
• Etoposide

Uncommon or rarely used drugs in adrenal cancer chemotherapy are:

• Paclitaxel (Taxol)
• 5-fluorouracil (5-FU)
• Vincristine (Oncovin)

Side effects of chemo-drugs and chemotherapy for adrenal cancer

The side effects on chemotherapy depend on the type of drug, dosage, and the duration of the treatment.

Common side effects of chemotherapy are:

• Hair loss
• Loss of appetite
• Fatigue, nausea and vomiting
• Skin problems (Hand and foot rashes)
• High or low blood pressure
• Increased risk of infection (deficiency of white blood cells)
• Problems with bleeding or bruising after minor cuts or injuries (due to a shortage of blood platelets)
• Anemia, or low red blood cell counts
• Mouth sores
• Diarrhea

Treatment choices by stage of adrenal cancer

The stages of adrenal cancer define the choice of treatment:

Stage I and II

Surgery is considered as the best treatment for the initial stages of adrenal cancer. The entire adrenal gland may be removed in some cases if the spread of the tumor is limited to the gland. As there are two adrenal glands in human body – one on each kidney, removal of any of the two won’t affect the body of the patient much.

If the person is treated with only drugs or radiations, a person has high chances for the adrenal cancer to reoccur.

An adjuvant therapy, which involves radiation or chemotherapy, may be given after the surgery to prevent the growth of cancer cells that remain in the body after the removal of the gland or other tissues. It also inhibits the reoccurrence of adrenal cancer.

Stage III

Surgery is considered as the main treatment method in this stage. The adrenal gland is removed with some parts of tissues of kidney and liver which are affected by the tumors cells of the adrenal cancer. The lymph nodes around the gland are also removed and adjuvant treatment with radiation and drugs (mitotane) are given to prevent the growth of tumor cells in future.

Stage IV

Debulking surgery may be done in this stage. In this surgery, the maximum affected parts or organs with the adrenal cancer are removed. This helps in lowering the hormone production level. This is the most severe stage of adrenal cancer where sometimes even surgery cannot cure the disease. The radiations also do not help cure the disease. The drugs might delay the symptoms of adrenal cancer but complete cure may not be possible at this stage. The objective of treatment is to help relieve the symptoms and improve the quality of life of the patient.

After the treatment of adrenal cancer

Even after the treatment of adrenal cancer, there is still a risk of its return which is common in all types of cancers. Therefore, regular follow-ups and screening as recommended by your healthcare practitioner are important. You must consider certain things after the treatment of cancer, as follows:

Follow up care

Regular checkups, routine visits to doctors, blood tests, etc are very important for every patient, especially in the first few years of the treatment. It reduces the risk of recurrence of the tumor and also help track the effect of treatment (and side effects) on other organs of the body.

Scans are done periodically and drugs are given to patients after the treatment if any sign or symptom is discovered for the recurrence of adrenal cancer.

Nutrition

Diet and physical activities are necessary for maintaining the body and to stay healthy. Most patients experience side effects of the treatment after the treatment of adrenal cancer is complete. You may experience a feeling of nausea, loss of appetite resulting in weight loss etc. Eating right food at regular intervals is important to maintain the nutrition level of the body.

Although, there are no dietary supplements which can reduce the risk of adrenal cancer (or any type of cancer), nutritional supplements which provide proper nutrition to the body and help the body stay healthy and keep the immune system strong can be taken.

Keeping away from tobacco and alcohol reduces the risk of adrenal cancer.

Keeping health insurance and copies of your medical records

Health insurance is important even after the treatment as regular follow-ups are also very costly. Even, in case of cancer returning back, you will require it.

Medical records must be kept safe as they help in the diagnosis of a person’s medical condition. Medical history helps the doctor understand a person’s condition and plan the treatment accordingly.

Recurrence of adrenal cancer: Second Cancers after adrenal cancer

The worst part of suffering with cancer is its reoccurrence even after getting the right treatment. There are many health and emotional problems a person can suffer with after the treatment of adrenal cancer. But, perhaps the worst of them is the fear of the coming back of the cancer. This coming back is termed as “reoccurrence” or “recurrence”. If this occurs, this is called “second cancer”.

Where does adrenal cancer spread to during metastasis?

The second cancer mainly affects the parts around the adrenal gland or the organ which were earlier affected during the first round of adrenal cancer. There is a high possibility of a person developing second cancers such as lung cancer, bladder cancer, skin cancer in women, prostate cancer. In a study, several patients were identified who suffered with acute leukemia, breast cancer, bone cancer, soft tissue sarcoma, genetic disease such as Li-Fraumeni syndrome, etc.

Can the risk of recurrence of adrenal cancer be reduced?

To help maintain good health and prevent reoccurrence of the adrenal cancer, cancer survivors should:

• Stay at a healthy weight
• Adopt and maintain a physically active lifestyle
• Eat a healthy diet, with an emphasis on plant foods and limit alcohol
• Consider regular follow-ups and checkups

People suffering with a recurrent adrenal gland tumor frequently experience emotions such as disbelief or fear as cancer limits their lifestyle and survival chances.

Adrenal cancer research: Clinical trials for adrenal cancer

Clinical trials establish ways to detect, reduce the risk, and treat cancer. Past clinical trials have led to:

• Formation of new and more effective drugs for specific types of cancer including adrenal cancer
• Rebuilding drugs for adrenal cancer with fewer side effects
• Better and more effective methods for diagnosis and treatment of adrenal cancer

New ways that are under research for the treatment of adrenal gland cancer:

Immunotherapy

Immunotherapy is also called biologic therapy. In this therapy, the body enhances its natural defenses to fight a tumor. Boosting the immune system with the help of either immune functions of the body or externally provided drugs is the objective of this therapy. Clinical trials are being done to improve the treatment methods of adrenal gland cancer.

Succinate dehydrogenase (SDH) tumors and molecular markers

SDH is a specialized protein that has an important role in our body’s metabolism. Researchers believe that when this gene is inactivated, it results in cancer, including adrenal gland tumors.

Whereas, molecular markers help in diagnosing the adrenal gland cancer at an early stage, these markers help in early treatment of the disease.

Palliative care

The primary goals of palliative care are to treat the symptoms such as nausea, vomiting, breathlessness, insomnia and other sleep-related problems, and other physical issues caused by the cancer or its treatment.

These diagnosis and treatment goals must be made clear to patients and their caregivers.

• Maintain the support for a patient’s emotional and social needs, spiritual needs, or other needs and concerns.
• Provide support to the caregivers, and other family members and friends of the cancer patient

Clinical trials are in progress to discover better ways of reducing the symptoms and side effects of current adrenal gland tumor treatments and to improve patients’ comfort and overall quality of life.

Adrenal cancer treatment failure: Is Stage 4 adrenal cancer terminal?

If the tumor has reached the advanced stage of adrenal cancer, the cure or recovery is not always possible. Advanced stage is often known as the terminal stage. The duration of survival can be limited to about six months.

Although, proper care and treatment is still provided to the patient, the cancer continues to spread all over the body at this terminal stage. This diminishes the life expectancy and survival significantly.