Adrenal Cancer

The adrenal gland is an endocrine gland that is present above the kidney and has got 2 parts the adrenal cortex and adrenal medulla which produce various hormones. The outer cortical layer produces cortisol and aldosterone whereas the inner medulla of the adrenal gland produces catecholamines which are dopamine, epinephrine, and norepinephrine. Cortisol helps to maintain the body’s reaction to stressful conditions. Aldosterone helps to maintain the salt-water balance in the body.

When there is an abnormal growth of the cells out of control forms a mass over the adrenal gland called an adrenal tumor. Adrenal tumors can be cancerous or noncancerous. The cancerous tumors grow in size and spread to the other parts of the body. Noncancerous tumors don’t grow in size. Adrenal cancer can be functional or nonfunctional. Functional tumors produce hormones whereas nonfunctional tumors don’t produce hormones. Less than 1% of adrenal tumors are cancerous.

There are so many risk factors for the development of adrenal cancers such as carney complex, Li-fraumani syndrome, neurofibromatosis type 1, MEN type 2, etc. diagnosis of adrenal cancer is mainly done on the history, clinical examination, and imaging tests are required to see the presence of the tumor. the main treatment is to remove the mass surgically. Chemotherapy, radiotherapy, and hormonal replacement are also done depending on the requirement.

Adrenal Cancer: Causes

The cause of adrenal cancer is unknown. But some of the important causes known to cause adrenal cancer include −

• Most adrenal cancers arise sporadically

• Some adrenal cancers occur as a part of familial cancer syndrome

• These can occur associated with hereditary tumor syndromes such as Li-fraumani syndrome, Familial adenomatous polyposis, Beckwith-Weidemann syndrome, Multiple endocrine neoplasia type 1, and neurofibromatosis 2.

• Medullary type of thyroid cancer

• Parathyroid hyperplasia

• Emotional and physical stress

• Increased or decreased secretion of hormones.

Adrenal Cancer: Symptoms

Symptoms of patients with adrenal cancer vary from person to person depending on the size, location, and spread of the tumor. The important symptoms of adrenal cancer include the following −

• Bruising

• Raised blood pressure due to the production of increased levels of hormones

• Weakness

• Raised blood sugar levels

• Reduced potassium levels and increased sodium levels can be present

• Increased or excessive hair growth

• Increased sweating especially more at the night

• Feels anxious or has panic attacks

• Weakness of the bones

• Palpitations

• Stretch marks on the abdomen

• Mood disturbances such as anxiety, depression, or irritability

• Nervousness

• Abdominal pain which can be present constantly or intermittently.

• Pain in the back, which may be localized or radiate to other areas of the body.

• Unexplained weight loss, which may be accompanied by a loss of appetite.

• Women may experience irregular periods, or they may stop menstruating altogether.

• Excess hair growth due to the production of excess hormones

• Enlarged lymph nodes. Adrenal cancer can cause lymph nodes to become enlarged, especially in the abdomen.

Adrenal Cancer: Risk Factors

Some of the patients having certain familial disorders are at increased risk of developing adrenal tumors. These include;

• Carney complex − It is a connective tissue disorder having high skin pigmentation, myxomas, and several adrenal gland tumors

• Li-Fraumeni syndrome − It is an autosomal dominant disorder

• Multiple endocrine neoplasia type 2 − It is a hereditary condition in which it comprises 3 primary tumors those are; medullary thyroid cancer, parathyroid tumors, and neuroendocrine tumor

• Neurofibromatosis type 1 − This condition presents with the growth of small tumor masses along the nerves and also presents with the pigmentation of the skin

• Familial adenomatous polyposis − It is a condition that presents with multiple colonic polyps, retinal lesions, thyroid carcinomas, and adrenocortical tumors.

• Beckwith-Weidmann syndrome

Adrenal Cancer: Diagnosis

The diagnosis of adrenal tumors is done based on the history, physical examination, and some of the investigations which include −

• Complete Blood count − Blood tests and urine tests are done to measure the number of adrenal hormones. Based on this the tumor can be differentiated as functional or nonfunctional.

• Clonidine suppression test − This test helps to differentiate between essential hypertension and pheochromocytoma

• Computed tomography

• MRI − The size of the tumor can be assessed using MRI

• Ultrasound helps to know the location and presence of the tumor

• Meta iodo benzyl guanidine scintigraph − This test helps to know the location of the tumor. The test is done in 2 days. On day 1 dye is injected. The pictures are taken after some hours and on the next day. If the tumor is not seen on an x-ray this test helps to diagnose cancer.

• A test for the levels of cortisol can be done. Urine may be collected for 24hrs.

• Biopsy is required to confirm the diagnosis. A sample of the tissue is taken and visualized under the microscope for cancer cells

• Adrenal vein sampling − In this, the blood from the vein of the adrenal gland is taken and tested to see the presence of the extra hormone from the cancer mass. This test is used when MRI or CT fails to diagnose the condition.

• Positron emission tomography scan may be required in some patients to look for the spread of the tumor

• Adrenal angiography

Adrenal Cancer: Treatment

The treatment of adrenal tumors is very important as it can lead to severe complications. Treatment of adrenal cancer can be done as follows;

• Hormone therapy is necessary before the surgery. If the tumor is cancerous then radiation therapy and chemotherapy should be done. Radiotherapy and chemotherapy are advised as adjunctive to the surgical removal or in patients who are not fit for surgery.

• Surgical removal of the tumor is the mainstay of the treatment. It is more effective in the early stages of adrenal cancer.

• Adrenal gland can be removed along with the removal of the involved lymph nodes

• Mitotane is the drug that is used to decrease the size of the tumor by blocking the excessive hormone production

• Alpha blockers: These act by blocking the catecholamine’s action on blood pressure

• Catecholamine synthesis inhibitors are used before the surgery

• Beta blockers like propranolol can be used

Adrenal Cancer: Prevention

There are no preventive measures to reduce the risk of adrenal cancers.

Conclusion

Adrenal gland cancers need to be assessed as the adrenal gland plays a very important role such as maintaining stress situations, and salt and water balance by producing various hormones required for the same. Adrenal cancers are the abnormal growth of cells excessively producing mass, these can function by actively secreting the hormones or can be silent without any secretion of the hormones.

Adrenal cancers are mainly sporadic and are associated with familial syndromes like carney complex, Li-fraumani syndrome, Familial adenomatous polyposis, Beckwith-Weidemann syndrome, Multiple endocrine neoplasia type 1, and neurofibromatosis type 2. Adrenal gland cancer presents various clinical symptoms such as raised blood pressure, weakness, anxiety, panic attacks, etc

Diagnosing adrenal cancer is very important as it can cause severe complications. The diagnosis is made based on the history, physical presentation, and investigations like blood tests, MRI, CT, and MIBG scintigraph, adrenal vein sampling. Once the diagnosis is made the treatment is done with hormone therapy, by removing the tumor surgically and drugs like mitotane, alpha-blockers, beta-blockers, and catecholamines are also given to treat the condition.