What Is the Main Idea?

Feminizing adrenocortical tumors are an extremely rare type of cancer that develops in the adrenal glands. In the open-access article “Feminizing Adrenocortical Tumor with Multiple Recurrences: A Case Report”, published in the journal Case Reports in Oncology, the authors describe the case of a 35-year-old man diagnosed with this type of cancer and how his treatment has been managed.

What Else Can You Learn?

Feminizing adrenocortical tumors and their symptoms are discussed, alongside the roles of case reports in raising awareness of rare conditions. The roles of the endocrine system and its key components, particularly the adrenal glands, are also described.

What Are Glands?

Glands are organs in the body that produce substances and release them either through ducts (openings) or directly into the bloodstream. Glands that release substances through ducts are called “exocrine” glands, and this group includes the glands that release milk, digestive juices, tears, and sweat.

“Endocrine” glands release hormones, molecules that act as chemical messengers, into the bloodstream. Together, hormones and endocrine glands make up the endocrine system, a messenger system that targets and regulates organs all over the body and controls almost all of the processes that take place within it.

What Does the Endocrine System Do?

To be able to function properly, the various parts of the body need to be able to communicate with each other to make sure that the internal environment is kept constant, and that any changes in the internal or external environment get an appropriate response. Two systems enable this communication:

  • The nervous system is made up of the nerves, spinal cord, and brain, and enables messages to travel from one part of the body to another within fractions of seconds.
  • In contrast, the endocrine system is better suited to responding to situations where a longer-lasting and more widespread response is needed, because it involves hormones being made and travelling around the body in the bloodstream.

Although the two systems complement and interact with each other, the endocrine system is responsible for regulating development, growth, metabolism (the process by which the food and drink that we consume is changed into energy), and our ability to reproduce, as well as the components that make up bodily fluids like saliva and blood, our emotions and moods, and even our sleep.

Which Parts of the Body Are Involved in the Endocrine System?

Although hormones are made in many parts of the body, there are several key components of the endocrine system. These include the pituitary and pineal glands and the hypothalamus in the brain, the thymus in the upper part of the chest, the thyroid and parathyroid glands in the neck, the pancreas (which is behind the stomach and is also part of the digestive system), the gonads (the “sex glands”: ovaries in women and testes in men), and the adrenal glands, which are located on top of the kidneys.

The production of hormones and their release must be tightly controlled to ensure that the body’s functions are regulated properly. To achieve this, many functions are regulated by several hormones that regulate each other via positive and negative feedback loops. For example, the effect of one hormone on an organ may cause that organ to release a second hormone that feeds back to the gland that sent the first hormone. This can prevent the message being sent by the first hormone from being “on” continuously.

What Do the Adrenal Glands Do?

The adrenal glands are triangular-shaped and there is one on each kidney. They are made up of two parts with different functions and that make different sets of hormones:

  • The inner part of the adrenal gland is called the “adrenal medulla”, and it is here that a type of hormone called “catecholamines” are made. The best known catecholamine is adrenaline (also known as epinephrine or the “fight or flight” hormone), which increases the body’s heart rate and blood pressure when it is under stress.
  • The outer part of the adrenal gland is called the adrenal cortex, and it is here that a type of hormone called “corticosteroids” are made. Some of the roles of these hormones include metabolism, the body’s response to stress, the immune system, and sexual development and function.

What Does This Case Report Describe?

A case report is a type of study that looks in depth at the case of a single individual or a specific group of patients. Case reports are particularly useful when healthcare practitioners want to communicate information about rare or previously unreported conditions, complications, or treatments to the rest of the medical community. In this study, the authors describe the case of a 35-year-old man who had a type of adrenal gland cancer called a “feminizing adrenocortical tumor”.

Primary tumors (tumors that have not spread from elsewhere in the body) that start in the tissues that cover your organs and glands can be classed as adenomas (these are “benign”, meaning that they are not able to invade surrounding tissue or spread to other areas of the body) or carcinomas (these are “malignant”, which means that they can invade and spread). Primary carcinomas of the adrenal glands are rare and, although it is unusual, sometimes a tumor in an adrenal gland can start to produce and release corticosteroids abnormally.

In the case of feminizing adrenocortical tumors, only estrogens are secreted. Estrogens are a type of sex hormone, so called because they are critical in regulating the biological differences between males and females, and are particularly involved in reproduction and puberty. In humans, the key sex hormones are estrogens, progesterone, and testosterone. The high levels of estrogens produced by feminizing adrenocortical tumors have a feedback effect on the levels of testosterone, meaning that testosterone production is usually suppressed in patients with this type of tumor.

As a result, common symptoms are hypogonadism (where the gonads produce low levels of or no hormones) and overdevelopment or enlargement of the breast tissue in men and boys. Patients with this type of tumor can also experience discomfort or pain in one side of the body between the back and the upper abdomen (belly area). Feminizing adrenocortical tumors most commonly occur in men but can also develop in women and children. In women, additional symptoms include irregular or postmenopausal bleeding.

One of the difficulties in treating feminizing adrenocortical tumors is that they are extremely rare, accounting for less than 2% of all adrenal gland tumors. In fact, only 50 cases were reported in the medical literature between 1970 and 2015. As a result, case reports have an important role to play in increasing awareness of this type of tumor and improving its diagnosis and treatment.

Take-Home Message

Feminizing adrenocortical tumors are often aggressive (meaning that they develop and/or spread quickly), are almost always malignant, and the chance that they will recur is high. Case reports like this study help to raise awareness of the need to recognize and treat this type of cancer aggressively, and monitor patients closely for signs of recurrence.

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