What Is an Adrenal Carcinoma?

The classification of adrenal tumors can be divided into benign tumors and malignant tumors according to their nature; non-functional tumors and functional tumors according to the presence or absence of endocrine function (such as secretion of a certain hormone causing hypertension); cortical tumors, Medullary tumors, stromal tumors, or metastases. Adrenal tumors that require clinical intervention are usually functional tumors or tumors that are highly suspected of being malignant (or benign and malignant cannot be identified before surgery).

Zhang Zheng	(Attending physician)	Department of Urology, Peking University First Hospital
Gong Kan	(Deputy Chief Physician)	Department of Urology, Peking University First Hospital

Western Medicine Name: Adrenal tumor

Affiliated Department: Surgery-urology
Contagious: Non-contagious

Overview of adrenal tumors

The adrenal gland is an important endocrine organ in the body. Due to its close relationship with the kidney, it is traditionally a urological disease. The human adrenal glands, one on the left and one on the right, are located behind the peritoneum. At first glance, the shape and size of the adrenal glands exactly resemble that of an adult's slightly flexed last finger. The head, body, and tail of the adrenal gland also look like the last finger from the root to the fingertips. However, if you carefully understand the part, the right adrenal gland is triangular. The left adrenal gland is half-moon shaped, the former is straddled on the inner side of the upper right pole of the right kidney, and the latter is suspended on the inner side of the upper left pole of the left kidney. Its length, width, and thickness are 4.0 cm ~ 6.0 cm, 2.0 cm ~ 3.0 cm, and 0.3 cm, respectively. ~ 0.6 cm. The normal adrenal gland weighs about 4.0 to 5.0 grams.

Although the volume of the adrenal gland itself is small, the volume of tumors that grow is very different. Usually, those with a diameter of less than 3 cm are called small tumors, the smallest is less than 1 cm, and the larger is more than ten to 30 cm. The shape of the tumor can be beans, peaches, apples, cantaloupe, baby pillow, etc.

Adrenal gland tumor physiological function

People have invested a lot of interest and energy in research in this field. Related biochemical and pharmacological researches have achieved rapid progress. We know that not only adrenaline can be obtained from the adrenal medulla, but also norepinephrine and dopamine, which have less side effects than adrenaline, and often have better blood pressure and life-saving effects. More importantly, for the function of the adrenal cortex, through nearly half a century of research, we know that there are more than 40 kinds of corticosteroids (chemical names: steroid hormones or steroids) produced and secreted from the adrenal cortex. Product or derivative. Up to 70 species. Corticosteroids can be roughly divided into three categories, briefly described as follows:

1. Corticosterone, the hormone that regulates sugar and protein metabolism, is represented by cortisol, and cortisone is commonly used in clinical practice. Such hormones promote amino acid deamination to sugar, that is, promote glycogen gluconeogenesis and maintain blood glucose concentration. Lack of this hormone is prone to hypoglycemia. When this hormone is too much, the glycogen heterogeneous effect is enhanced, which can destroy the protein or prevent its synthesis, causing the human subcutaneous fat to increase excessively, the blood sugar to rise, the skin becoming thinner, purple lines, muscle weakness, and osteoporosis. In addition, glucocorticoids have an effect on the metabolism of various substances. Together with insulin, auxin, and adrenomedullin, they can regulate the body's material metabolism and energy supply, and coordinate and balance physiological activities in the body.

2. Mineralocorticoid, a hormone that regulates salt and water metabolism, is represented by aldosterone, and its clinical application is deoxycorticosterone acetate. This hormone causes the renal tubules to absorb sodium and chlorine and excrete potassium and phosphorus. Without this hormone, the concentration of sodium in the plasma decreases, so water is lost, blood is concentrated, and blood potassium is increased. Too much of this hormone is the opposite, with increased blood sodium and decreased potassium. Mineralocorticoids can also have a certain effect on the metabolism of sugar and protein, but the effect is lighter. The production and secretion of mineralocorticoids are mainly regulated by the renin-angiotensin system under physiological conditions, followed by the effects of serum potassium and adrenocorticotropic hormones.

3 The sex hormone adrenal cortex also secretes weaker male hormones such as dihydroepiandrosterone, androstenedione and trace amounts of testosterone, which do not play an important role in adult marriage and childbirth. However, adolescent boys and girls can contribute to the earliest secondary sexual characteristics such as the emergence of axillary hair and pubic hair, as well as the maturity of the hypothalamus-pituitary-gonadal axis, thereby enabling healthy development during adolescence. The adrenal cortex also secretes a small amount of estrogen, which is generally of no practical significance, but in patients with adrenal tumors, due to its increased content, it can cause impotence and infertility in male patients, and menstrual disorders in female patients.

These corticosteroids in the human body through the hypothalamus |-pituitary-adrenal axis of the regulatory energy and neurohumoral feedback system function, their secretion most often changes with the day and night hours, the rhythm changes, so the hormone in the blood of the human body The content also changes with time. For example, the content of cortisol is highest at 8-9 o'clock in the morning, and the lowest is around 24 o'clock at midnight. This can maintain the normal and orderly progress of the human body's metabolism, growth and development, and physiological activities; and when encountering an unexpected emergency situation, that is, when the human body can be suddenly stimulated or hit by some kind of spirit, such as when encountering a certain kind of Enormous difficulties must be overcome immediately, surrounded by siege, brave breakthroughs, tenacious competition want to knock down the opposition, dystocia, major surgery, major bleeding must be alive, etc., even afterwards, they are often inexplicable: how can you be anxious at that critical moment , How can there be that extraordinary perseverance, how can there be such a big life immortality. In fact, this is also due to the adrenocortical hormone secretion also has a stress-fixing characteristic. Experiments show that during major surgery and major bleeding, cortisol levels can increase several times or even ten times, and at the same time, through the negative feedback regulation mechanism, the pituitary gland can be released to promote the release of adrenocorticotropic hormones, which enhances the body's stress ability and abnormal ability.

Once a tumor occurs in a certain part of the adrenal gland, the hormone in the corresponding part will be excessively secreted, the so-called functional adrenal tumor, causing a series of clinical symptoms related to excessive hormone secretion.

Functional adrenal tumors common to adrenal tumors

Adrenal tumor cortisol

A series of pathophysiological changes and clinical manifestations caused by increased cortisol in the human body are cortisol, or hypercortisol, formerly known as Cushing's syndrome.

1. Cause

(1) Due to the presence of adrenal tumors (adenomas or cancers), there is an excessive excess of cortisol secretion; approximately 25% of cases.

(2) Due to the presence of pituitary adenomas or disorders of the hypothalamus and even the central nervous system, the pituitary gland secretes too much adrenocorticotropic hormone, causes bilateral adrenal hyperplasia, and secretes too much corticosteroids.

(3) Due to organ tumors other than the endocrine system (such as small cell lung cancer), carcinoid tumors (lung, gastrointestinal), thymoma, pancreatic tumor, medullary thyroid cancer, ganglion tumor, melanoma and prostate cancer, etc. During the onset, ACTH (as ectopic ACTH syndrome) is increased.

(4) Increase in iatrogenic corticosteroids. Due to the long-term heavy use of glucocorticoids, similar symptoms appeared, and gradually disappeared after stopping treatment.

2. symptom

This disease is mostly female. From 1980 to the end of 1998, the Cancer Hospital of the Chinese Academy of Medical Sciences treated a total of 48 cases, 30 females and 18 males, aged 12 to 74 years. The patients were mostly obese and their limbs were not fat. The so-called "concentric obesity." Bald head, round face, the so-called "full moon face" is crimson and dark with sores, and there is a lot of fat behind the neck and shoulders, such as "buffalo back". The skin is thin, hairy, armpits, both sides of the lower abdomen, and purple stripes The patient had high blood pressure, complained of general weakness, and waist and leg pain. Female patients developed typical symptoms such as dumbness, amenorrhea or menstrual disorders, and osteoporosis.

3 diagnosis

Generally, it is not difficult. As long as you have common sense in this regard and increase your vigilance against the disease, remember the symptoms and signs mentioned above at a glance, because many of her (his) symptoms are on the face of. Based on this clue, blood is drawn and urine is collected for endocrine hormone tests. B-ultrasound, CT, and MBI are used for tumor localization tests to confirm the diagnosis. Because such cases are rare, even ordinary medical personnel rarely see them, it is inevitable that they will be misdiagnosed. In addition to the routine blood and urine test items for patients with cortisol, plasma cortisol, 24-hour urine free cortisol, 24-hour urine 17-hydroxycorticosteroids, and 24-hour uremia 17-ketosteroids must be measured. If the plasma cortisol at 8:00 am in the morning exceeds 138 micromoles / or higher than 10 g / dl, it can be diagnosed as hypercortisolism. On the eve of taking blood

At 11:00, oral dexamethasone 1mg is used to inhibit hypothalamic-pituitary-adrenal function. In addition, the blood ACTH value can be measured, which is suppressed below the normal value in patients with adrenal tumors. Its normal value is 20 ~ 100g / dl. Cortisol patients with other causes of increased ACTH in blood are not suppressed.

In addition, the size and nature of adrenal tumors and their relationship to surrounding structures must be examined by B-ultrasound, CT, or MRI, as well as X-ray radiographs, tomography, and X-ray radiographs of the skull saddle. Radiographs, tomography and 3D saddle radiography, as well as CT scans, magnetic resonance imaging to diagnose the presence or absence of pituitary adenomas or microadenomas.

4. Treatment is mainly based on the cause. For patients with adrenal tumors, try to remove the tumor as much as possible, especially for benign tumors, the effect is positive. For malignant tumors, tumor resection is used to relieve symptoms, followed by other adjuvant treatments to improve quality of life and prolong survival. Palliative treatments such as radiation, chemotherapy, and immunotherapy should be given to those who are too poor for surgical treatment or extensive transfer. For patients diagnosed with pituitary tumors, sphenoidal pituitary tumor resection through the nose is effective. After excision of the primary tumor in patients with ectopic ACTH syndrome, ACTH can gradually fade away, and those who cannot control can use adrenal enzyme inhibitors such as memitotane, aminohypnosis, or drugs that directly act on the hypothalamus and pituitary such as cemetidine , Bromocriptine and other controls.

Adrenal tumor aldosterone

Aldosterone can be divided into two types: primary and secondary. Primary aldosterone is a rare disease that is caused by excessive secretion of aldosterone in adrenocortical adenomas and hyperplasia. Secondary aldosteronism is caused by various extra-adrenal diseases caused by excessive aldosterone secretion, including nephrotic syndrome with varying degrees of edema, cirrhosis, ascites, heart failure, and aggressive type of hypertension. Described herein is primary aldosterone disease.

Primary aldosterone syndrome is a syndrome in which renin secretion is suppressed due to an increase in aldosterone secretion in the human body. It is clinically characterized by hypertension and hypokalemia. In 1954, Kong reported the first case of adrenocortical adenoma that secreted aldosterone and healed. This disease was named Conn's syndrome. The adrenal glands that cause this disease are mostly small benign adenomas, located in the outermost layer of the adrenal glands. Adrenal cancer is rare, accounting for about 1% of low renin aldosterone. Even less than 50 cases have been reported internationally. At present, it is estimated that primary aldosterone disease accounts for 0.65% to 2% of hypertension

1. symptom

(1) Hypertension is mainly due to increased blood volume and increased vascular resistance caused by increased sodium ions, which is the main or earliest symptom of this disease. Increased blood pressure is moderate or slightly severe. Malignant hypertension can occur in children, with a maximum pressure of 34.5 / 20.5kPa. Antihypertensive drugs are generally not effective. There is also a normotensive aldosteronism, the mechanism of which is unknown. Dizziness, headache, fatigue, blurred vision, upset, thirst and other symptoms are often caused by hypertension and hypernatremia.

(2) Hypokalemia Hypokalemia causes muscle weakness and muscle paralysis, which makes patients feel light-headed, weak limbs, lower limbs are more obvious, and severe cases show periodic paralysis. Hypokalemia leads to arrhythmia, cerebral hypoxia syndrome, and polyuria and nocturia due to renal dysfunction. When affecting the pancreas, fasting blood glucose increases.

(3) Alkaline poisoning caused by imbalance of water and electrolyte balance will eventually lead to the loss of calcium and magnesium ions, numbness of the extremities and spasm of pain in the extremities.

2. diagnosis

(1) In children or adolescents with hypertension, a corresponding examination should be made in consideration of the possibility of the disease.

(2) In adults with hypertension, the effect of taking antihypertensive drugs is not obvious, and those with hypokalemia or periodic lower extremity paralysis should consider this disease for further examination.

(3) Laboratory inspection

Measure the concentration of potassium and sodium in plasma and the amount of potassium excretion in 24 hours. Hypokalemia is spontaneous or prone to occur, or coexisting hypokalemia, the disease should be highly suspected.

Measure the plasma or 24-hour urine aldosterone concentration and plasma renin activity. Plasma renin activity was lower than 2.46molL / h in standing position, and the ratio of plasma aldosterone concentration to plasma renin activity was> 20 in standing position.

The aldosterone inhibition test was negative. The aldosterone secretion of ortho-aldosis is autonomous. This rule out primary hypertension and secondary aldosterone.

The secretion and excretion of glucocorticoids are the most normal.

Oral sodium chloride inhibition test: Plasma aldosterone level is above 554pmll / L, urinary aldosterone value is above 38.8nmol / 24h, and urine sodium excretion exceeds 200mol / 24, which can be diagnosed as protoaldehyde.

Laboratory test results, such as hypertensive patients with normal glucocorticoid secretion, increased aldosterone secretion cannot be suppressed by a high-sodium diet, accompanied by spontaneous hypokalemia and increased urinary potassium excretion, can be diagnosed as protoaldehyde.

(4) Imaging diagnosis

In addition to adrenal adenomas and adenocarcinomas, primary adrenal hyperplasia also accounts for a large proportion. The former is mainly treated by surgery, and the latter must be treated with drugs. The two methods are different, and the three must be differentially diagnosed by B ultrasound, CT, and MRI. Because the adenomas that cause orthoalgia may be very small, dense scans with a 0.5 cm spacing can be used for CT scans to avoid missing tumors. When identification is difficult, an adrenal isotope iodide cholesterol scintillation scan plus dexamethasone inhibition test can be used, that is, after the patient is injected with 131I-6iodomethyl-19 nornorcholesterol and scanned. Cortical adenomas absorb more radiation than normal. Markers, normal cortical hyperplasia uptake, cortical cancer was not shown. Its accuracy can reach 70% ~ 90%.

3 Adrenal tumors are mainly treated with surgery. For smaller adenomas, enucleation is generally used, and normal tissues at a distance of 0.5 cm around the tumor are removed together. Due to polyploid abnormalities in the adrenal tissue close to the tumor, tumor recurrence can be caused. After surgery, the serum potassium and aldosterone values of the adenoma cases returned to normal, and the symptoms disappeared. The literature of adenocarcinoma cases all considered the prognosis to be poor. Drug treatment: The spironolactone microparticle antitisone 120 mg 3 times a day and amlodipine 5 mg 3 times a day, alone or in combination, can return blood potassium and blood pressure to normal. Or antihypertensive drugs such as Xintongding can be used in combination with antihistone.

Adrenal gland tumor

The abnormality of the external genitalia and sexual characteristics caused by some congenital or acquired diseases of the kidney is called adrenal abnormality or adrenal reproductive syndrome.

1. Classification of this disease is also rare, with about a hundred cases reported in China. This disease can be named according to the age of onset, gender, etiology, and type of abnormality.

2. Etiology and hyperplasia occur mainly in the cortex-related areas. The sex hormones produced and secreted by the human adrenal glands account for the majority, and estrogen is scarce. During the normal development of the adrenal cortex, the normal function of the enzyme must be present in order to complete it successfully. The lack of enzymes or the obstacle of the function, that is, affect the synthesis of cortinin, promote the proliferation of the adrenal cortex, and thus increase the effect of androgens. , A large number of male corticosteroids convert female patients to males. The cause of the tumor is due to the tumor's secretion and accumulation of more sex hormones in the body.

3 Symptoms Adrenal abnormalities are mainly manifested in the conversion of female patients to males. The so-called transformation of the sex is just the change of the genital shape, and its true sex has not changed, because the gonads and sex chromosomes that determine its sex have not changed. Therefore, the so-called "female pseudohermaphroditism" that occurs during the fetal period indicates that she is different from the true hermaphroditism, which has both ovaries and testes in the body, and there are very few such cases. Female pseudohermaphroditism can see the clitoris, labia majora, and external genitalia of the baby boy with congenital hypospadias at birth. The author has seen an untreated girl with a voice and a smile similar to that of a boy with skin. Dark, hairy, dumb, clitoris like penis, can erect, big labia like scrotum. The urethra is the opening of the genitourinary sinus. The abnormality of the sexual characteristics of the male infant during the fetal period is mainly the larger external genitalia, and then it grows rapidly. The physical size of the 4-5 year old child and the external genitalia are as large as right. Those born with normal prepubertal disease at birth are mostly caused by adrenal tumors. The main symptoms are: disappearance of subcutaneous fat, virilization of the body, hypertrophy of the clitoris, low voice, shrinking of breasts and uterus, stop of menstruation, and loss of libido.

4 diagnosis

(1) First, check the type and local condition of the abnormality of the clear signs by physical examination for reference of deformity correction.

(2) If necessary, perform an adrenal scan of dexamethasone inhibition test through image diagnosis to identify whether it is a hyperplasia, adenoma or cancer.

(3) For hyperplasia and those with Cushing's syndrome, the contents of 17 ketones, 17 hydroxyls, 21 hydroxylases, and 11 hydroxylases in urine for 24 hours should be checked in detail.

(4) Find out the true gender of the patient, pay attention to the identification with the male hypospadias combined with cryptorchidism, male pseudohermaphroditism, true hermaphroditism, mixed gonadal dysplasia, etc. If necessary, do a chromosome test or laparotomy.

5. treatment

Patients with tumors must be resected. The larger ones are resected together with the surrounding tendons and, if necessary, the kidneys. The basic principle for the treatment of patients with hyperplasia is to supplement the lack of cortisol, inhibit the excessive secretion of ACTH of the pituitary gland to stop the proliferation and hypertrophy of the adrenal cortex, reduce the excessive secretion of androgen, and relieve or alleviate virilization.

Adrenal medullary tumor

Adrenal tumor pheochromocytoma

The adrenal medulla is in the middle of the adrenal gland and only accounts for about 10% of the adrenal gland. The morphology of medulla cells is different. Because when the medulla cells are treated with a liquid containing chromium, the particles in these cells can be colored, so they are called chromaffin cells.

Pheochromocytomas are mostly benign, accounting for approximately 90%. Therefore, its shape is smaller than , or as big as cantaloupe. Generally the size of a tangerine, it is oblate and slightly fan-shaped, and the cut surface is dark yellow or brown. It has a leafy structure, tumor cells are irregularly polygonal, small or large, and the bones are multinucleated and contain most of the chromaffin-stained particles, especially those suspected of malignancy are dark and dark for reference.

Adrenal medulla. Sympathetic nerve endings and the central nervous system can synthesize dopamine, norepinephrine, and epinephrine from blood chromine as raw materials. These three are collectively called catecholamines. In the sympathetic nervous system and the central nervous system, catechol is synthesized by nerve cells and then transmitted to nerve endings for release. Dopamine in the adrenal medulla is an intermediate product. It must be converted into norepinephrine and further into adrenaline by the action of dopamine beta-enhancing enzyme. Both can be directly released into the blood circulation. When a pheochromocytoma develops a tumor, a large amount of epinephrine and norepinephrine are stored in the tumor. It is not easy to be noticed by patients themselves or others at ordinary times, but once encountering some kind of stimulus, the tumor releases a considerable amount of catecholamines, the patient's blood pressure will rise suddenly, the heart rhythm will be disturbed, and an explosive blow will be encountered. Blow.

symptom

Most of the patients are young adults aged 20 to 40, and the ratio of male to female is almost equal. The main symptom is changes in basal metabolism of hypertension: hypertension can be paroxysmal or persistent, or paroxysmal exacerbations of persistent hypertension. Persistent persons often have dizziness, headache, chest tightness, chest pain, heartbeat, palpitation, blurred vision, nervousness, anxiety, and fear of heat. Paroxysmal patients have sudden severe headache, palpitations, chest tightness, pale, sweating, shortness of breath, and the patient feels near death. At this time, if the blood pressure can reach 40.OkPa (200 ~ 300Hg), it may relieve itself after about half an hour. After recovery, as usual. After encountering some kind of irritation again. The number of gradual attacks is more frequent, the interval is shorter, and the situation is getting worse. Seizure irritation may not be very strong. Some people may have awakening, sweating, or dying when they rinse their teeth or dream. There are also tumors that are huge and have high blood pressure and have no onset symptoms, or have no lumps, have no attacks, and died of surgery for other diseases. Therefore, patients with such symptoms should be checked, diagnosed and treated as soon as possible.

diagnosis

According to literature statistics reports, if there are five typical symptoms, abdominal mass, hypertension, diabetes, and increased basal metabolism, the presence of pheochromocytoma should be suspected. Three of the five are highly suspected, and four of them can be diagnosed. undoubtedly. Among them, the most important thing is to find a lump. If the tumor is small and located outside the adrenal glands, it must be qualitative. Position the two sides to start the inspection. Qualitatively, urine norepinephrine, epinephrine, and urine 3-methoxy-4-hydroxymandelic acid (VMA) were measured. Its normal value is 8 to 165 micrograms / 24 hours. Determination of catecholamines in plasma binding state. If the blood catecholamines are measured in a resting supine position, the value of catecholamines can be diagnosed by combining with the clinic. If the dopamine value increases, often the surface tumor is malignant. Its accuracy is better than measuring the catecholamine value of the free state of blood. Orientation: B-ultrasound, CT, MRI can generally be used to diagnose adrenal tumors, hyperplasia, bleeding, medullolipoma, etc. Without this equipment, retroperitoneal gas injection can still be used to locate small pheochromocytoma outside the adrenal gland. There may still be difficulties. Segmented blood drawn from the inferior vena cava can be used to detect blood plasma catecholamines or abdominal aorta angiography for clues. If possible, use the radionuclide iodobenzidine (131I MIBG) tracer scan for -ray photography. More special effects.

treatment

Pheochromocytoma should be surgically resected, because it is mostly benign tumors, and most of them have good postoperative results. However, the risks of surgery and anesthesia are greater, especially for large tumors with abundant blood vessels and close to the surrounding large blood vessels, which are prone to bleeding; tumors contain a large amount of catecholamines, which are easily squeezed into the blood, causing the patient's blood pressure to spike and heartbeat Sudden stop. After the advent of phenoxybenzamine and propranolol in the 1970s, they were taken by patients before surgery. They were prepared to block adrenergic receptors for 2 to 4 weeks, relieved fluctuations in blood pressure and heart rhythm during surgery, and improved the safety of surgery. It is still required to fully prepare before surgery and postoperative care, and gently operate during the operation to ensure the successful completion of the operation.

Adrenal malignant tumor

Adrenocortical carcinomas are rare and generally functional. They are generally larger than adenomas when found, often weighing more than 100g, showing infiltrative growth, normal adrenal tissues are damaged or submerged, and they invade surrounding adipose tissues. Lateral kidney. Small adenocarcinomas can have an envelope. The cut surface is brownish yellow, and bleeding, necrosis, and cystic changes are common. Microscopically poorly differentiated patients have high atypia, tumor cells of varying sizes, and odd-shaped nuclei and multiple nuclei can be seen, and mitotic images are more common. Often metastasized to abdominal aortic lymph nodes or hematogenous metastases to the lungs, liver, etc. A highly differentiated person looks like an adenoma under the microscope. If the cancerous body is small and has an envelope, it is difficult to distinguish it from an adenoma. Some people think that those with a diameter of more than 3cm should be considered as a highly differentiated adenocarcinoma.

Adrenal tumor disease treatment

Laparoscopic surgery has become the most common way to remove adrenal tumors. The advantages are obvious. First, it is minimally invasive, that is, only a few holes with a diameter of 1cm can be used to complete the tumor resection. The postoperative recovery is very fast. The traditional open surgical incision is more than ten centimeters, which allows the patient to recover. Slow, affecting aesthetics; second, clarity. Due to the magnifying effect of laparoscopy, a very deep adrenal gland is close to the eyes, achieving a clear field of vision unmatched by open surgery, coupled with the use of advanced cutting and separating instruments, making surgery The anatomy is quite fine with minimal bleeding. ^[1]