Adrenal cancer

Overview

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)