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Adrenal cancer, also called adrenocortical carcinoma (ACC), is a form of cancer that originates in the outer layer of the adrenal glands (cortex). The adrenal glands exist as a pair, with one positioned on top of each kidney. These glands play a crucial role in producing hormones that regulate the functioning of various organs and tissues throughout the body.
Cortisol, testosterone, aldosterone, and estrogen are all produced by the adrenal cortex. These hormones regulate male and female characteristics as well as bodily processes such as blood pressure and stress response.
Adrenocortical carcinoma is classified into two types: functioning ACC tumor which triggers the adrenal gland to produce more hormones than it normally would, and nonfunctioning ACC tumors which have no effect on hormone production.
Most adrenal gland growths are noncancerous. Adrenal tumors, such as adenoma or pheochromocytoma, can also form in the adrenal glands. Adrenal cancer is a rare type of cancer that can develop at any age. However, it is more likely to affect children under the age of five and individuals in their 40s and 50s.
Timely detection of adrenal cancer significantly improves the chances of survival. Early detection of adrenal cancer increases the possibility of a cure. However, if the cancer has metastasized beyond the adrenal glands, the likelihood of a complete cure diminishes. In such cases, treatment can be employed to postpone the advancement of the disease or prevent its return.
Symptoms
The signs and symptoms of adrenal cancer may manifest in different ways. When an adrenal cortical carcinoma (ACC) is actively producing hormones, it can lead to symptoms associated with hormone overproduction. If the adrenal tumor becomes large enough, it may push on other organs nearby. This pressure might result in discomfort and other symptoms.
Depending on the cause, adrenocortical cancer symptoms may include:
- Abdominal pain and bloating
- High blood pressure
- High blood sugar levels
- Unexplained weight gain, particularly in the face, neck, and trunk, but not in the arms and legs
- Early puberty in both boys and girls
- Hormonal changes in women may result in increased facial hair, hair loss on the scalp, and irregular periods
- Gynecomastia or excessive breast growth in boys or men
- Unexplained weight loss
- Muscle weakness
- Stretch marks that are pink or purple on the skin
- Nausea and vomiting
- Back ache
- Fever
Causes
It is believed that people who are born with particular genetic disorders are predisposed to adrenal cancer. However, the exact cause of this condition is still unknown.
The exact mechanisms behind the genetic mutations that lead to adrenocortical cancer remain uncertain according to experts. Typically, gene mutations can occur spontaneously within cells, independent of inheritance. Within a cell’s DNA, there exist instructions that guide its functioning. Mutations can disrupt this process, causing cells to grow uncontrollably and survive when healthy cells would normally die. As a result, these abnormal cells can aggregate and form a tumor. Subsequently, tumor cells have the potential to detach and spread to other parts of the body. The development of adrenal cancer arises when changes affect the DNA of a cell within the adrenal gland.
Risk factors
Certain inherited genetic disorders can increase the likelihood of developing adrenal cancer. In around 50% of cases, adrenocortical cancer has a familial tendency. Several genetic disorders are associated with an increased risk of developing this type of cancer. These include:
- Beckwith-Wiedemann syndrome
- Carney complex
- FAP or familial adenomatous polyposis
- Li-Fraumeni syndrome
- Lynch syndrome
- Multiple endocrine neoplasia, type 1 (MEN 1)
- Type 1 neurofibromatosis (NF1)
- Von Hippel-Lindau syndrome (VHL)
Diagnosis
The diagnosis of adrenal cancer often begins with a physical examination and an assessment of any symptoms the patient has. To confirm the diagnosis, several tests may be necessary, such as:
- Blood and urine tests: These tests can measure the hormone levels in the body, specifically those generated by the adrenal glands, such as cortisol, aldosterone, androgens. Unusual levels may be linked to adrenal cancer.
- Imaging tests: MRI, CT scan, or PET scan are used to detect malignancies. Imaging tests can help the doctor clarify any growths on the adrenal glands. This also can determine whether cancer has spread to other parts of the body, such as the lung or liver.
- Biopsy: A biopsy is used to assess whether a tumor is malignant and which sorts of cells are involved. The doctor may advise to have the afflicted adrenal gland removed for laboratory examination.
Treatment
There are several available treatment options for adrenal cancer. Typically, when a tumor is identified as malignant, the doctor will assess the extent of the disease, including measuring the size of the tumor and determining if cancer cells have spread beyond the adrenal gland to other areas of the body. This information is crucial for determining the appropriate course of treatment.
Depending on whether the cancer is on the early or advance stage, the following treatment options may be recommended:
- Surgery: The common first treatment for adrenal cancer is surgery. Adrenalectomy entails removing the adrenal gland and possibly other adjacent tissue as well. If evidence of cancer spreading to neighboring structures is discovered, such as the liver or kidney, sections or all those organs may be removed during the procedure.
- Medication to lower the risk of recurrence: Although still under research, Mitotane may be used after surgery for patients who are at high risk of cancer recurrence. This medication is used to treat advanced adrenal cancer and has shown promise in slowing disease recurrence following surgery.
- Radiation therapy: Radiation therapy kills cancer cells by delivering high-energy beams such as X-rays and protons. Radiation therapy is commonly performed following adrenal cancer surgery to eliminate any remaining cells. It can also aid in the relief of pain and other symptoms associated with cancer that has spread to other regions of the body.
- Chemotherapy: Chemotherapy may be an option to reduce the progression of adrenal tumors that cannot be removed surgically or that return after initial treatments. Chemotherapy uses drugs to destroy cancer cells and prevent tumor growth.
