What is adrenal tumor?
Adrenal tumors can be classified into benign and malignant tumors according to their nature; non-functional and functional tumors according to the presence or absence of endocrine function; and cortical tumors, medullary tumors, mesenchymal tumors or metastases according to the site of occurrence. Adrenal tumors that require clinical intervention are usually functional tumors or tumors with high suspicion of malignancy (or those that cannot be identified as benign or malignant before surgery).
The adrenal gland is an important endocrine organ in the body and is traditionally a urological disease due to its location in close relationship with the kidneys. The human adrenal glands, one on the left and one on the right, are located behind the peritoneum, while their lower lateral side is in close proximity to the upper medial side of both kidneys. Although the adrenal glands themselves are small in size, the size of the tumors that grow in them varies greatly, with those less than 3 cm in diameter being called small tumors, the smallest being less than 1 cm, and the largest being more than 10 to 30 cm. The shape of tumor can be like bean, peach, plum, apple, cantaloupe, child’s pillow, etc.
Physiological functions of the adrenal glands
Much interest and effort has been devoted to research in this area. Research on the biochemical and pharmacological aspects of the gland has made rapid progress. We know that not only adrenaline, but also norepinephrine and dopamine can be obtained from the adrenal medulla, and their side effects are less than those of adrenaline, and their effect of raising blood pressure and saving lives is often better. More importantly, in terms of adrenal cortex function, through nearly half a century of research, we know that there are more than 40 kinds of corticosteroids (chemically called steroid hormones or steroids) produced and secreted from the adrenal cortex, and if we add some available intermediate products or derivatives. There can be more than 70 species.
Corticosteroids can be roughly divided into three categories, which are briefly described as follows.
1. Hormones that regulate sugar and protein metabolism. Corticosteroids are represented by cortisol, and cortisone is commonly used in clinical practice. These hormones promote amino acid deamination into glycogen, that is, they promote glycogen xenobiotic effect and maintain the concentration of blood sugar. Lack of this hormone makes hypoglycemia easy to occur. When there are too many of these hormones, gluconeogenesis is enhanced, which can destroy proteins or prevent their synthesis, resulting in excessive increase in subcutaneous fat, elevated blood glucose, thinning of skin with purple lines, muscle weakness and osteoporosis. In addition, glucocorticoids have an effect on the metabolism of various substances, which together with insulin, growth hormone and adrenomedullary hormone regulate the metabolism of substances and energy supply of the body, so that the physiological activities in the body are coordinated and balanced with each other.
2.Hormones that regulate salt and water metabolism. Salt corticosteroid is represented by aldosterone, and the clinical applicator is deoxycorticosterone acetate. This hormone makes the renal aqueduct absorb sodium and chloride while excreting potassium and phosphorus. If there is a lack of this hormone, the concentration of sodium in plasma decreases, thus water is lost and blood is concentrated, while blood potassium increases. If there is too much of this hormone, the situation is reversed, i.e. sodium increases and potassium decreases. Salt corticosteroids can also have some effect on the metabolism of sugar and protein, but the effect is less severe. The production and secretion of salt corticosteroids are mainly regulated by the renin-angiotensin system in the physiological state, followed by the influence of blood potassium and adrenocorticotropic hormone.
3. Sex hormones. The adrenal cortex also secretes weak androgens such as gliadin androstenedione, androstenedione and trace amounts of testosterone, which do not play an important role in the married life of adults. However, it can contribute to the appearance of the earliest secondary sexual characteristics, such as axillary and pubic hair, and the maturation of the hypothalamic-pituitary-gonadal axis in adolescents of both sexes, thus enabling healthy pubertal development. The adrenal cortex also secretes trace amounts of estrogen, which is generally of no practical significance, but in patients with adrenal tumors, its increased levels can cause impotence and infertility in male patients and menstrual disorders in female patients.
When there is major surgery and bleeding, cortisol level can rise several times or even more than ten times, while promoting the release of pituitary gland pro-adrenocorticotropic hormone through a negative feedback regulatory mechanism, enhancing the body’s stress capacity and supernormal ability. Once a tumor occurs in one part of the adrenal gland, the hormone in the corresponding part will be overproduced, which is called functional adrenal tumor, causing a series of clinical symptoms related to hormone overproduction.
Functional adrenal tumor cortisolism
A series of pathophysiological changes and clinical manifestations caused by increased cortisol in human body is cortisolism, or cortisolism, formerly known as Cushing’s syndrome.
1. Etiology.
(1) Due to the presence of adrenal tumors (adenoma or carcinoma), the autonomic secretion of excessive cortisol; about 25% of cases.
(2) Due to the presence of pituitary adenoma or hypothalamic or even central nervous regulation disorder, the pituitary gland secretes too much pro-adrenocorticotropic hormone, causing bilateral adrenal cortex hyperplasia and excessive secretion of corticosteroids.
(3) Increased autocrine secretion of ACTH (in the form of ectopic ACTH syndrome) during the pathogenesis of organ tumors outside the endocrine system (such as small cell lung cancer), carcinoid tumors (lung, gastrointestinal), thymoma, pancreatic tumor, medullary thyroid-like carcinoma, ganglion tumors, melanoma and prostate cancer.
(4) Increased medical-derived corticosteroids. Similar symptoms appear due to long-term heavy glucocorticoid treatment and gradually disappear after stopping the drug.
2. Symptoms.
This syndrome is predominantly female, and patients are mostly obese in the body, but not in the limbs. That is the so-called “centripetal obesity”. Bald head, round face, the so-called “full-moon face” deep red obscure with sores, the back of the neck and shoulders more fat, such as “buffalo back”, thin skin, hairy, armpits, lower abdomen on both sides, the femur has purple lines, the patient more high blood pressure, complaining of general weakness, back and leg pain. Female patients appear silent, amenorrhea or menstrual disorders, osteoporosis and other typical symptoms.
3.Treatment.
The main treatment is to deal with the cause of the disease. Patients with adrenal tumors should strive to remove the tumor as much as possible, especially for benign tumors, whose efficacy is certain. For malignant tumors, also to remove the tumor to relieve the symptoms, followed by other adjuvant treatment to improve the quality of survival and prolong the survival period. For those whose general condition is too poor for surgical treatment or extensive metastasis, palliative treatment such as radiation, chemotherapy and immunotherapy will be given. For those diagnosed with pituitary tumors, the results are good after transsphenoidal saddle pituitary tumor resection. In cases of ectopic ACTH syndrome, ACTH can gradually subside after resection of the primary tumor. Those who cannot be controlled can be controlled with adrenal enzyme inhibitors such as albuterol, aminoglutethimide, or drugs that act directly on the hypothalamus and pituitary gland such as cephaeline and bromocriptine.
Aldosteronism
Aldosteronism can be divided into two types: primary and secondary. Primary aldosteronism is a rare disease caused by excessive secretion of aldosterone from adrenal cortical adenomas, hyperplasia and other lesions. Secondary aldosteronism is caused by aldosterone overproduction from various extra-adrenal diseases, including nephrotic syndrome with varying degrees of edema, cirrhosis with ascites, heart failure, and hypertension in the acute form. Primary aldosteronism is described in this article.
Primary aldosteronism is a syndrome in which renin secretion is suppressed due to increased secretion of aldosterone in the body, and is characterized clinically by hypertension and hypokalemia. 1954, Kong first reported a case in which an aldosterone-secreting adrenocortical adenoma was removed and cured, thus naming the disease Conn’s syndrome. The majority of adrenal glands that cause this disease are small benign adenomas located in the outermost layer of the adrenal gland. Adrenal carcinoma is rare, accounting for about 1% of cases of hyporeninogenic aldosteronism. Even less than 50 cases have been reported internationally. Current international estimates suggest that primary aldosteronism accounts for 0.65%-2% of hypertension
1, symptoms.
(1) Hypertension is mainly due to increased plasma volume and increased vascular resistance caused by increased sodium ions, and is the most dominant or earliest symptom of the disease. Blood pressure increases to moderate or slightly severe levels. In children, a malignant form of hypertension may occur, with a maximum pressure of 34.5/20.5 kPa. There is also normotensive prodromalgia, the mechanism of which is unknown. Due to hypertension and high blood sodium often cause dizziness, headache, fatigue, blurred vision, irritability, thirst and other symptoms.
(2) Hypokalemia hypokalemia causes muscle weakness and muscle paralysis, causing the patient to feel light-headed and weak in the limbs, more pronounced in the lower limbs, and in severe cases presenting periodic paralysis. Hypokalemia leads to cardiac arrhythmias, cerebral hypoxia syndrome, and polyuria and nocturia due to renal dysfunction. When the pancreas is affected, fasting blood glucose is increased.
(3) Water and electrolyte balance imbalance causes alkalosis, which eventually leads to the loss of calcium and magnesium ions, and the right presents numbness of the extremities, pain in the extremities must spasm.
2, diagnosis.
(1) In children or table juvenile hypertensive patients, the possibility of this disease should be considered and the corresponding examination.
(2) In adult hypertensive patients who take antihypertensive drugs with no obvious effect, accompanied by hypokalemia or periodic lower extremity limb paralysis, this disease should be considered for further examination.
(3) Diagnosis by imaging
In addition to adrenal adenoma and adenocarcinoma, adrenal cortical hyperplasia also accounts for a large proportion of patients with proaldosteronism. The former is mainly treated by surgery, while the latter must be treated by medication. The two methods are different, and differential diagnosis must be made by ultrasound, CT and MRI. Since the adenoma causing prodromalgia may be very small, CT scan with a dense layer of 0.5cm spacing can avoid missing the tumor. When difficulties are encountered in differentiation, adrenal isotope iodinated cholesterol scintigraphy with dexamethasone suppression test can be applied, i.e. the patient is scanned after the injection of 131I-6β iodomethyl-19 desmethyl cholesterol, the cortical adenoma absorbs more amount of radiomarkers than normal, the cortical hyperplasia uptake is normal, and the cortical cancer is not shown. Its accuracy rate can reach 70%~90%.
3.Treatment. Adrenal tumor is mainly treated by surgery. For smaller adenomas, enucleation is generally used, together with the normal tissues around the tumor at a distance of 0.5cm, because the adrenal tissue close to the tumor has polyploidy abnormalities, which can cause tumor recurrence. In adenoma cases, the blood potassium and aldosterone values return to normal after surgery and the symptoms disappear; in adenocarcinoma cases, the prognosis is considered poor in the literature. Pharmacological treatment: The particulate form of spirolactone, Atixotone 120mg 3 times daily and Amclopramide 5mg 3 times daily can be used alone or in combination to normalize the blood potassium and blood pressure. Or use antihypertensive drugs such as cardiac painkillers in combination with antiseptic.
Adrenal Sexual Abnormalities
Abnormalities of the external genitalia and sexual characteristics caused by a congenital or acquired disease of the kidney are called adrenal gonadal syndrome or adrenal reproductive syndrome.
1. Classification.
This syndrome is also relatively rare, about a hundred cases have been reported in China. This syndrome can be named according to the age of onset, gender, etiology, and type of abnormal sex characteristics.
2. Etiology.
The cause of the disease is hyperplasia, which occurs mainly in the cortical network related aspects of the belt. The human adrenal glands produce and secrete mainly sex hormones and very little estrogen. In the process of normal development of adrenal cortex, there must be normal action of enzymes in order to complete smoothly. The lack of supply of enzymes or obstruction of action affects the synthesis of corticotropin, which prompts the hyperplasia of adrenal cortex, thus increasing the action of androgens, which is equivalent to adding fuel to the fire, and a large amount of androgenic corticotropin transforms female patients into males. If the disease is caused by tumor, it is due to more sex hormone components secreted and accumulated in the body by the tumor.
3. Symptoms.
Abnormal adrenal sex characteristics are mainly manifested in the transformation of female patients to males. The so-called sex transformation is only a change in the appearance of the genitalia, but the real sex remains unchanged, because the gonads and sex chromosomes that determine their sex remain unchanged. Therefore, the so-called “female pseudohermaphroditism” that occurs during fetal life indicates that it is different from true hermaphroditism, in which both gonads are present in the ovaries and testes, and such cases are rare. Female pseudohermaphroditism can be seen at birth with the clitoris and labia majora shaped like the external genitalia of a male infant with congenital hypospadias. The authors have seen a case of an untreated girl with the same appearance as a boy, with dark skin, hairy and reticent, with a clitoris like a penis, erect, and labia majora like a scrotum. Its urethra is the opening of the urogenital sinus. The abnormal sexual characteristics of male infants in the fetal period are mainly manifested by larger external genitalia, and later they grow rapidly, and children aged 4 to 5 years have physical and external genitalia right as into from size. The onset of normal prepubertal period at birth, its cause is mostly caused by adrenal tumors. The main symptoms are: disappearance of subcutaneous fat, masculinization of body physique, enlarged clitoris, low voice, shrinking of breast and uterus, cessation of menstruation, loss of libido, etc.
4.Diagnosis
(1) Firstly, the type of abnormal sexual characteristics and local conditions should be checked by physical examination for reference of deformity correction surgery.
(2) Diagnose by imaging, and if necessary, perform adrenal scan by dexamethasone suppression test to identify whether it is hyperplasia, adenoma or cancer.
(3) In case of hyperplasia and Cushing’s syndrome, the 24-hour urine levels of 17 ketones, 17 hydroxyl, 21 hydroxylase, 11 hydroxylase, etc. should be checked in detail.
(4) Find out the real gender of the patient, pay attention to differentiate from male severe hypospadias combined with cryptorchidism, male pseudohermaphroditism, true hermaphroditism, mixed gonadal dysplasia, etc. If necessary, do chromosome examination, or dissection.
5.Treatment
Tumor patients should have their tumors removed, and the larger ones should be removed together with the surrounding tendons and, if necessary, the kidneys. The basic principle of treatment for patients with hyperplasia is to supplement the lack of cortisol, inhibit the overproduction of pituitary ACTH to stop the hyperplasia and hypertrophy of the adrenal cortex, and reduce the overproduction of androgens to relieve or alleviate the masculinization.
Adrenal medullary tumor pheochromocytoma
The adrenal medulla is in the middle part of the adrenal gland and accounts for only about 10% of the adrenal gland. The morphology of the medullary cells varies, and they are called chromophobe cells because the granules in these cells are found to be colorable when the medullary cells are treated with chromium-containing fluid.
Pheochromocytomas are mostly benign, accounting for about 90% of cases. Therefore, its shape is either smaller than a loquat or as large as a cantaloupe. Generally, it is the size of a mandarin and is flat and slightly scalloped, with a dark yellow or brown surface. The tumor cells are irregularly polygonal, either small or large, with multinucleated cytoskeletons and contain most chromophobic staining particles, especially dark and dark in those suspected to be malignant.
Adrenal medulla.
Both sympathetic nerve endings and the central nervous system synthesize dopamine, norepinephrine and epinephrine from chromogenic acid in the blood, which are collectively known as catecholamines. In both the sympathetic nervous system and the central nervous system, catecholamines are synthesized by nerve cells and then passed to nerve terminals for release. Dopamine in the adrenal medulla is an intermediate product that must be converted to norepinephrine and further to epinephrine by the action of dopamine beta-enhancerase, both of which can be released directly into the circulation. When chromophobe tumors occur, there is a large amount of epinephrine and norepinephrine stored in the tumor. However, once the tumor releases a considerable amount of catecholamines in response to a certain stimulus, the patient will experience a sudden increase in blood pressure and heart rhythm disorder, and will experience an explosive or even fatal blow.
Symptoms.
The main symptom is hypertension drinking changes in basal metabolism: hypertension can be paroxysmal or persistent, or persistent hypertension intensifies paroxysmally. Persistent people usually have dizziness, headache, chest tightness, chest pain, heartbeat panic, blurred vision, mental tension, anxiety, fear of heat, etc. In paroxysmal cases, there is a sudden severe headache, palpitation, chest tightness, pale face, sweating, shortness of breath, and the patient has a feeling of near death. At this time, if the blood pressure is measured up to 40.OkPa, about half an hour or so after the possible self-remission. After recovery, a normal person. Later on, when encountering some kind of stimulus, the attack will occur again. Gradually, the number of seizures becomes more frequent, the interval is shortened, and the situation becomes heavier. The stimulus of seizure may not be very strong. There are cases when the seizure wakes up during gargling and brushing teeth or in dream, sweating profusely and having a sense of near death. There are also patients with huge tumors and high blood pressure who never have seizure symptoms, or those who die during surgery for other diseases without a mass or seizure. Therefore, patients with such symptoms should be examined and treated early.
Treatment.
Pheochromocytoma should be surgically removed because it is mostly a benign tumor and most of them have good postoperative results. However, surgery and anesthesia are more dangerous, especially for large tumors, which are rich in blood vessels and close to the surrounding large blood vessels, so they are easy to bleed; tumors contain a lot of catecholamines, which are easily released into the blood by extrusion, causing a sharp rise in blood pressure and cardiac arrest. However, it still requires adequate preoperative preparation and postoperative care, and gentle operation during surgery to ensure the smooth and successful completion of surgery.
Adrenal malignant tumor
Adrenocortical carcinoma is rare, usually functional, and is usually larger than adenoma when found, often weighing more than 100g. It grows infiltratively, destroying or flooding normal adrenal tissue and invading outward to the surrounding adipose tissue or even the kidney on that side. Smaller adenocarcinomas may have an envelope. The cut surface is brownish-yellow in color, and hemorrhage, necrosis and cystic changes are common. Microscopically, poorly differentiated adenocarcinoma is highly heterogeneous, and the tumor cells are of different sizes, with odd-shaped nuclei and multinuclei. Metastasis to abdominal aortic lymph nodes or hematogenous metastasis to lung and liver is common. If the cancer is small and has envelope, it is difficult to distinguish it from adenoma. Some people think that if the diameter is more than 3 cm, it should be considered as highly differentiated adenocarcinoma.
Treatment of disease
Currently, laparoscopic surgery has become the most common way to remove adrenal tumors. The advantages of laparoscopy are obvious: firstly, it is minimally invasive, i.e. only a few small holes of 1 cm in diameter are needed to remove the tumor and the postoperative recovery is fast, while the incision in traditional open surgery is more than 10 cm, which makes the patient’s postoperative recovery slow and affects the aesthetics. The use of advanced cutting and separating instruments makes the surgical dissection quite fine and bleeding minimal.