Adrenal Tumors (Adrenal Gland Tumors)

The adrenal glands, in other words, the adrenal glands, are a pair of endocrine organs located in the upper pole of both kidneys, yellow-green in color, each weighing 4-5 g, measuring 5x3x1 cm, and secreting some hormones that the body needs, which do not have a direct relationship with the kidneys except in their anatomical neighborhood. The adrenal glands consist of two parts, the outer shell (cortex-80%) and the inner (medulla-20%) part. The outer shell consists of three layers, and different hormones are secreted from each layer. These hormones are:

1-Aldosterone: It is secreted from the outermost layer (glomerulosa) of the shell (cortex) part of the adrenal gland and tries to maintain the water and salt balance of the body by affecting the kidneys. It helps to keep blood pressure balanced in the body by controlling sodium and potassium levels.

2-Cortisol:It is secreted from the middle layer (Fasiculata) of the shell (cortex) part of the adrenal gland and affects the metabolism and contributes to the body’s coping with stress and infections.

3- Dihydroxy Epiandrosterone (DHEA):It is secreted from the innermost region of the shell (cortex) part of the adrenal gland and contributes to the shaping and maintenance of gender characters (hair and hair distribution, gender-appropriate body) by acting as an androgen in men and like estrogen in women.

4-Catecholamines: In the inner-Medulla part of the adrenal gland, hormones called catecholamines (epinephrine-adrenaline, norepinephrine and dopamine) are secreted especially in situations of fear, stress and effort. These increase the heart rate, increase the blood pressure, increase the respiratory rate, raise tension and awareness to the upper level and enable the body to get rid of dangerous situations and manage this situation.

Classification of Adrenal Tumors : Adrenal gland tumors are divided into two groups as benign and malignant (cancer), and they are divided into two subgroups according to whether they produce excessive hormones (functional and non-functional). In other words, these tumors do not always mean cancer, they are mostly benign tumors.

1-Benign tumors:Benign adrenal tumors are not cancerous, do not spread (metastasize) to other sites and organs, refer to relatively small tumors (adenoma), and are discovered incidentally in check-up or screening tests (Adrenal Incidentaloma).

2-Functional tumors (excess hormone-producing): These tumors are also mostly benign, and a very small number of them are malignant adrenal cancers. They produce hormones excessively and patients come with a clinical picture according to the type of hormone they produce excessively. Functional adrenal tumors usually overproduce a single hormone, although in rare cases they can overproduce more than one type of hormone.

3-Malignant adrenal cancers: Adrenal cancers are rare (4-12/million people), and they are usually adrenocortical tumors originating from the shell. It is more genetically risky and appears to be associated with some syndromes.

Causes and risk factors of Adrenal Tumors: The causes of adrenal tumors are not fully understood, but some rare genetic conditions increase the risk, particularly the risk of developing adrenal cancer. These include multiendocrine neoplasia type 2 (MEN-2), von Hippel-Lindau syndrome (VHL), familial paraganglioma syndrome, neurofibromatosis type 1, Carney complex and Li – Fraumeni syndrome.

Symptoms of Adrenal Tumors: Tumors that do not produce hormones and/or are small in size may not usually cause any symptoms. Symptoms of adrenal tumors vary depending on the type of tumor and the hormones it secretes. Common symptoms include an increase in blood pressure, unexplained weight gain, feeling thirsty, and frequent urination; low potassium levels, heart palpitations, extreme irritability, anxiety or panic attacks, headache, excessive sweating, diabetes, abdominal pain, weakness and weakness, cracks in the abdominal skin and excessive hair growth can be seen.

Adrenal Tumor Types

1-Accidental Adrenal Tumors (incidentalomas): These tumors refer to tumors that we encounter incidentally in routine health screenings and controls, without any symptoms, and these constitute the majority of adrenal tumors. About 5% of people over the age of 50 who undergo routine or other reasons CT or MRI are diagnosed with a small adrenal cortex tumor. While most (90%) are benign, a small percentage are malignant (cancer) or functional (producing excess hormones) and may have tumors that need to be surgically removed. High blood pressure is the most common symptom of incidental tumors. Other symptoms include facial flushing, heart palpitations, muscle weakness and anxiety.

2-Aldosteronomas (Conn Syndrome): It is a disease of the outermost layer (glomerulosa) of the shell (cortex) part of the adrenal gland and is a benign tumor characterized by excessive secretion of aldosterone, it is called Conn’s syndrome or hyperaldosteronism. Aldosterone is a hormone that raises blood pressure due to water-salt retention in the kidneys and lowers potassium levels. In their tumors, aldosterone is secreted excessively and causes fatigue, bloating, intense thirst and frequent urination, and low potassium and high salt (sodium) levels in the blood, in addition to uncontrolled high blood pressure. Sometimes, the same picture, namely hyperaldosteronism-Conn’s disease, can be seen due to the excessive work of this bilateral layer (Bilateral adrenal hyperplasia-BAH) even without a tumor. If the necessary treatments are not provided, high blood pressure can cause stroke, heart attack and kidney failure.

3–Adrenal Cushing’s Syndrome: Cushing’s syndrome is a clinical picture caused by the high cortisol produced by the adrenal gland due to the tumor or overwork of the middle layer (Fasiculata) in the shell. Cortisol is a hormone that affects metabolism and under normal conditions, it supports metabolic events that will enable the body to protect the body and resist these stresses in cases of stress such as surgery, injury or infection. Hypersecretory cortisol in tumor or bilateral adrenal hyperplasia with overproduction; While it causes fat accumulation-central lubrication especially on the body and face, it does not cause any change in the arms and legs. This phenotypic changes are typical for Cushing’s syndrome, resulting in asymmetrical fat deposition and rapid weight gain on the face and a buffalo hump-buffalo hump on the shoulders and back. Apart from these findings, again due to excessive secretion of cortisol; thinning of the skin, typical purple stretch marks (Stretch) in the abdomen and thighs, increased facial hair and excessive hair growth, restlessness, extreme nervousness and depression, weakness and fatigue, thinning and weakness in the muscles, sexual dysfunction, osteoporosis can be seen. It also causes type2-diabetes, low potassium levels and hypertension. Menstrual disorders can be seen in women.

4-Sex Hormone Producing Tumors: It is a very rare disease, which is the clinical picture caused by the high sex hormone-androsterone (DHEA) produced by the tumor of the inner layer (Reticularis) in the shell (cortex) part of the adrenal gland or due to its overwork. Excessive production of sex hormones-androgens; It causes early puberty in young men, deepening of the voice in women, increased hair growth and facial acne and menstrual disorders. These tumors were found to be more likely to be malignant-cancer than other adrenal tumors. Again, tumors that secrete both sex hormone-androgen and other adrenal hormones are more likely to be malignant-cancer.

5-Peochromocytoma and Paragangliomas: Pheocrocytoma refers to the clinical picture caused by high catecholamines (epinephrine-adrenaline, norepinephrine and dopamine) produced due to tumor or overwork of the inner (Medulla) part of the adrenal gland. Normally, these hormones help the body cope with stress again and keep the body protected and alert to any danger. However, in tumors or hyperplasia (overproduction state) where these hormones are over-secreted, it causes high blood pressure that cannot be controlled by drugs and can lead to serious conditions such as stroke-stroke. There is even a risk of sudden death. 90% of pheocrocytomas are benign tumors and only 10% are malignant-cancers. Paragangliomas are a type of neuroendocrine (hormone-producing) tumors found in the abdomen, pelvis, chest, and neck. Genetic diseases such as multiple endocrine neoplasia and von Hippel-Lindau disease increase the risk of developing pheochromocytoma and other adrenal tumors. Clinically in pheochromocytoma and paragangliomas

sweating, headache, high blood pressure, increased heart rate and palpitations, excessive anxiety and fear, and increased blood sugar can be seen.

6-Adrenal Cancers: Only 5-10% of tumors of the adrenal gland are malignant-cancers. Adrenal cancers make up only 00.5%-02% of all cancers, they are very rare cancers. It most commonly occurs under the age of 5 and in the age group of 40-50. These cancers have a very severe and aggressive course, spread rapidly, metastasize to other regions and organs, and cause many complaints and findings in the patient. Depending on the hormone secreted by cancer, its spread and direct effect, patients may experience weight gain, hypertension due to fluid retention, swelling and edema, diabetes, abnormal growth of hair, hair growth, and abnormal skin changes. Depending on the area where the cancer licks and metastasizes, abdominal pain, a feeling of fullness and weight loss may occur.

Diagnosis of adrenal tumors: Advanced technology is generally used to diagnose adrenal gland tumors. Anamnesis, systematic physical examination and blood pressure and body measurements (weight, height, etc.), routine laboratory tests, hormone levels and some complex hormone level measurements determine whether there is hormone above normal and abnormal with imaging tests such as CT, MRI and whole body PET scans. tumor is detected. A biopsy can help confirm the diagnosis, if needed. In a tumor detected by imaging methods, the first thing to do is to check whether the tumor is functional or not, that is, whether it produces excess hormones. Then, this mass can be distinguished from benign or malignant-cancer. For all these:

Blood and Urine Tests: Blood or urine tests, some simple and complex hormone levels measurement, are performed to check for abnormal hormone levels in the body that may be produced by adrenal tumors. In some cases, 24-hour urine collection and measurements of the level of adrenal hormone wastes excreted in this urine may be required. In these laboratory tests, blood potassium, sodium, renin levels are important in diagnosing aldosteronoma. Measurement of cortisol level, one of the adrenal hormones, is important in the diagnosis and high cortisol levels may indicate the presence of Cushing’s syndrome. Blood Dopamine, norepinephrine and epinephrine are measured and the wastes of these hormones (methanephrine) are measured in the urine, and their high levels may be a sign of pheochromocytoma. Again, if an androgen-producing adrenal tumor is suspected, it can test the levels of adrenal androgen such as dehydroepiandrosterone, also known as DHEA, in the blood, or 17-ketosteroid, which is a waste (metabolite) in the urine for 24 hours.

Display Methods: If high adrenal hormone levels suggest an adrenal tumor, imaging tests are used to confirm the diagnosis. The vast majority of adrenal tumors occur incidentally (incidentaloma) during imaging tests performed for other medical reasons or by routine controls.

Ultrasonography:Tumors larger than 1 cm can be detected in routine ultrasonography. It is a very easy and inexpensive method. It is accepted as a diagnostic tool.

CT scan(Computed tomography):A tomography with contrast material can provide a clear view for adrenal tumors. X-rays are used.

Magnetic Resonance Imaging (MR): In this imaging method, an idea about the structure of the mass is obtained. It is important in terms of showing the prevalence of this tumor in adrenal cancers. Radio waves are used in this method. Again, contrast material is used.

PET Scan: This radioisotope nuclear medicine examination, which is important for cancer diagnosis, shows us whether the adrenal tumor is cancerous or not, whether it has metastasized, or whether another known cancer has spread to the adrenal gland. It also gives an idea of ​​how intense and active the cancer is.

Adrenal Venous Sampling:A stent is inserted through a large vein in the thigh, allowing blood samples to be taken from both adrenal and renal veins. This is done to determine if excessive hormone production is due to tumor, or overwork of the two adrenal tissues by looking at the level of aldosterone and renin from the blood.

Treatment of adrenal tumors:

1-Active Monitoring and Control: Adrenal masses smaller than 4 cm that are not functional, that is, do not produce hormones and do not show any suspicion of cancer in imaging methods, are usually included in the control protocol with active monitoring only once every 6-12 months. Because tumors smaller than 4 cm are less likely to be cancer. Some masses may require more frequent controls. Controls are performed at regular intervals (6-12 months) using routine blood, urine, hormone levels and imaging methods.

2-Surgery: Adrenal tumors that are functional, that is, cause serious complaints due to excessive hormone production or are larger than 4 cm, are surgically removed. It is protected from serious long-term health problems such as uncontrollable high blood pressure, heart attack, weight gain, diabetes and kidney problems. Since adrenal masses larger than 4 cm have a high probability of being cancer, surgical treatment is still required whether they are functional or not. Surgery either removes only the entire adrenal gland mass or removes the entire adrenal gland together with the mass.

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