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Adrenal gland cancer

Located above the kidneys, the adrenal glands play a significant role in the body's functioning by producing hormones such as adrenaline, noradrenaline, aldosterone, cortisol, testosterone, and estrogen. Masses can develop in the adrenal glands that are responsible for the production of these hormones. These masses, also referred to as adrenal tumors, can be either benign or malignant. Adrenal gland cancer, or adrenal cortex cancer, predominantly affects children under the age of 5 and individuals in their 40s and 50s. The exact cause of adrenal gland cancer, which can be treatable with early diagnosis, is not fully understood.

Symptoms of adrenal gland cancer include unexplained weight loss or gain, muscle weakness, nausea, vomiting, and the development of stretch marks on the abdomen. Treatment options for adrenal gland cancer encompass surgical intervention, radiotherapy, chemotherapy, and medication-based therapies. The Department of Urology of Uniqacare Clinic provided information about adrenal gland cancer.

What is adrenal gland cancer (pheochromocytoma)?


Adrenal gland cancer (adrenal cancer) is a rare cancer that begins in one of the small, triangular-shaped glands (adrenal glands) located above the kidneys. The adrenal glands produce hormones that instruct nearly every organ and tissue in the body. While it can occur at any age, adrenal gland cancer predominantly affects children under the age of 5 and individuals in their 40s and 50s. Early diagnosis allows for effective treatment of adrenal gland cancer, but the chances of successful treatment decrease when the cancer has metastasized.


Most tumors that form in the adrenal glands are not cancerous; they can also be benign tumors. Benign tumors such as adenomas or pheochromocytomas can develop in the adrenal glands. Determining whether a tumor in the adrenal gland is cancerous or benign is often challenging. Benign tumors do not spread, while cancerous tumors can spread to lymph nodes or other areas of the body.


Tumors that originate in the adrenal glands can be categorized as follows:


  1. Adenoma: This is the most common type of adrenal gland tumor. It is not cancerous; rather, it's a benign mass.

  2. Adrenal Cortical Cancer (Adrenal Cortical Carcinoma): This is a rare tumor, but it constitutes the most common type of cancerous adrenal gland tumor.

  3. Pheochromocytoma: This is a tumor that originates in the hormone-producing part (medulla) of the adrenal glands. In most cases, it is not cancerous.

  4. Neuroblastoma: This cancerous tumor most frequently starts in the adrenal glands in children. However, it can also originate in the neck, chest, or spinal cord.


Why does a mass or tumor form in the adrenal gland?


The exact causes of adrenal gland cancer are not fully understood. Although not definitive, studies suggest that mutations occurring in DNA might lead to the transformation of cells in the adrenal gland into cancerous ones. It is presumed that adrenal gland cancer arises when mutations occur in the DNA of an adrenal gland cell. Tumor cells can break apart and spread to other areas of the body.


What are the symptoms of adrenal gland cancer?


The question "how is adrenal gland cancer recognized" is among the most frequently asked questions. Symptoms of adrenal gland cancer are often caused by the hormones produced by the tumor. Some symptoms emerge when the tumor is quite large and exerts pressure on nearby organs.


Signs and symptoms of adrenal gland tumors include:


  • Unexplained weight gain or weight loss

  • Muscle weakness

  • Red to purplish-pink stretch marks on the skin

  • Hormonal changes that can lead to increased facial hair, hair loss on the head, and menstrual irregularities in women

  • Hormonal changes that can cause breast tissue enlargement and testicular shrinking in men

  • Nausea, vomiting

  • Abdominal or back pain

  • Fever

  • Loss of appetite

  • Formation of stretch marks on the abdomen

  • Muscle cramps

  • Headaches

  • Feeling of fullness in the abdomen or feeling full after eating only a small amount

  • Sexual problems

  • Fatty areas on the shoulders and the back of the neck

  • Low potassium levels

  • Anxiety or new panic attacks

  • High blood pressure

  • Diabetes


How is adrenal gland cancer detected? How is it diagnosed?


To diagnose adrenal gland cancer, a specialist physician first performs a physical examination. After obtaining the patient's medical history and listening to their symptoms, if there is a suspicion of cancer, certain tests need to be conducted. These tests can be listed as follows:


Blood and Urine Tests: These tests detect unusual hormone levels produced by the adrenal glands, such as cortisol, aldosterone, and androgens, in the blood and urine.

Imaging Tests: Imaging tests like MRI, CT scan, and PET-CT are used to detect any growth in the adrenal glands and to determine whether the tumor has spread to other parts of the body.

Biopsy and Pathology Tests: If there is suspicion of adrenal gland cancer, a biopsy sample is taken and analyzed pathologically to provide information about the content of the tumor.


What are the treatment options for adrenal gland cancer?


The treatment for adrenal gland cancer can vary based on the type of cancer, test results, and the stage of the cancer. The goal of treatment can be to cure the patient, control the cancer depending on its stage, and alleviate the discomfort caused by the cancer. Typically, the treatment of this disease involves surgery to remove the entire cancer.


Surgery: The primary treatment for adrenal gland cancer is the removal of the adrenal gland through a procedure called adrenalectomy. The surgeon aims to remove the cancer, including any areas it may have spread to. Nearby lymph nodes must be checked for spread and removed if necessary. While one approach involves an incision on the back to remove the adrenal gland, this method can be challenging for viewing large tumors. As a result, the surgery is usually performed through the front of the abdomen. This method provides better visualization of the tumor and its potential spread. In cases of metastasized cancer, other organs may also need to be partially or entirely removed during this procedure.


For small adrenal tumors, laparoscopic removal is also possible. While laparoscopic surgery can be used to treat benign tumors, it's generally not suitable for larger adrenal gland cancers. This is because these cancers often need to be removed in fragments rather than as a whole, which could increase the risk of cancer spread.


Radiation therapy: Radiation therapy is not typically used as the primary treatment for adrenal cancer, as killing cancer cells with x-rays can be challenging. However, radiation therapy can sometimes be used to kill remaining cancer cells after adrenal cancer surgery or to prevent the tumor from returning. This is known as "adjuvant" therapy. Radiation can also be used to treat areas where cancer has spread, like bones or the brain, and to help reduce cancer symptoms.


Chemotherapy: Chemotherapy involves using specific types of drugs to kill cancer cells. Typically, these drugs are administered intravenously or orally (in pill form). These drugs enter the bloodstream and can be effective for cancers that have spread (metastasized) beyond the adrenal gland, reaching other parts of the body. For adrenal cancers that cannot be removed surgically or return after initial treatments, chemotherapy can be an option to slow down cancer progression.


Use of medications to reduce the risk of recurrence: After surgically removing all visible cancer, patients might receive adjuvant therapy for a period to target any remaining microscopic cells.


Medications to control hormone levels: Often, patients need hormone replacements after adrenal gland removal. These medications can be used to block hormones produced by the cancer or reduce the effects of hormones. Treatment with some of these medications should be overseen by an endocrinologist.


FREQUENTLY ASKED QUESTIONS ABOUT ADRENAL GLAND CANCER


What is the adrenal gland? What is the adrenal gland?


The adrenal glands are small glands located above each kidney. These glands are small and yellowish in color, typically weighing around 10 grams. They constitute one of the body's endocrine system components, which consists of hormone-producing tissues and organs. Hormones are chemical substances transported through the bloodstream that affect the activities of other organs or cells in a specific way.

The adrenal glands consist of two main parts:

  • Adrenal Cortex: This is the name for the outer part of the adrenal gland. The cortex produces three main hormones: cortisol, aldosterone, and dehydroepiandrosterone (DHEA). Most adrenal cancers originate in this region.

  • Cortisol: It helps the body cope with stress.

  • Aldosterone: It regulates the amount of salt in the blood and blood pressure.

  • Adrenal androgens: These hormones can convert into sex hormones like estrogen and testosterone in other parts of the body. However, the amount of these hormones produced in the adrenal glands is relatively small compared to what is produced in other parts of the body. Testes produce androgens (male hormones) in males, while ovaries produce estrogen (female hormones) in females.

  • Adrenal Medulla: The inner part of the adrenal glands is called the medulla. Medulla, an extension of the nervous system, produces three hormones named epinephrine, norepinephrine, and dopamine. Often referred to as catecholamines, these hormones regulate the body's responses to stress, including controlling adrenaline levels.


What are the stages of adrenal gland cancer?

After a definitive diagnosis of cancer is made through a biopsy, efforts are made to determine the stage of the tumor. Determining the stage is crucial for planning treatment. Following the tests and diagnostic methods used to determine whether the cancer has spread to other parts of the body, the staging of the disease is carried out.

Early adrenal gland cancer: This refers to Stage 1 or Stage 2. In this stage, the tumor is still small in size and has not spread beyond the adrenal gland.

Advanced adrenal gland cancer: Stage 3 and Stage 4 adrenal gland cancer indicates that the tumor has spread to other areas of the body. Cancer cells often spread to nearby lymph nodes first. Advanced cancer can also spread to other organs, tissues, blood vessels, and bones.


Can adrenal gland cancer be prevented?

Since the exact cause of adrenal gland tumors is not fully understood, there is no specific preventive measure in place. However, if there is a history of adrenal gland tumors in a family member, it is recommended that other family members undergo genetic testing.


What are the risk factors for adrenal gland cancer?

Experts do not fully understand what causes adrenal cancer. However, certain factors are believed to increase the risk of adrenal gland cancer. These include specific genetic syndromes and a family history of the disease. Risk factors for adrenal gland cancer, also known as adrenal cancer, can be listed as follows:

  • Li-Fraumeni syndrome

  • Beckwith-Wiedemann syndrome

  • Von Hippel-Lindau syndrome

  • Multiple endocrine neoplasia (MEN) type 1 and 2

  • Familial adenomatous polyposis (FAP)

  • Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC)

  • Neurofibromatosis type 1

  • Carney complex


What are the complications of untreated adrenal gland cancer?


Untreated adrenal gland tumors can continue to grow and exert pressure on other organs. This pressure can impact the functioning and well-being of those organs and can also lead to various symptoms. Functioning adrenocortical carcinomas, which are cancers that produce excessive hormones, can cause various complications. Different hormones result in different symptoms. The excessive production of hormones and their effects are outlined below:


Excessive cortisol production can lead to Cushing's syndrome and the following symptoms:

  • High blood pressure

  • Elevated blood sugar levels

  • Weight gain in the face, neck, and trunk

  • A fat pad between the shoulders

  • Hair growth on the face and body

Excessive aldosterone production can lead to Conn's syndrome and the following symptoms:

  • Extreme thirst

  • High blood pressure

  • Cramps or muscle weakness

  • Frequent need to urinate

High testosterone production in females can result in:

  • Hair growth on the face and body

  • Deepening of the voice

  • Cessation of menstruation

High estrogen production in females can result in:

  • Irregular menstrual bleeding

  • Postmenopausal bleeding

  • Weight gain

High estrogen production in males can result in:

  • Decreased sexual desire

  • Sexual dysfunction

  • Breast enlargement


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