adrenal medulla hyperfunction
Introduction
Introduction to adrenal medulla hyperfunction Adrenal medullary tumors - pheochromocytoma is the main cause of medullary hyperfunction, and now clinically confirmed cases of medulla hyperplasia leading to medullary hyperfunction. Cases of pheochromocytoma have been reported as early as the 1st century, but successful reports of clinical surgical removal of tumors have been reported since 1927. In 1949, Holton and 1950, Goldenberg discovered that pheochromocytoma secretes adrenaline and norepinephrine. Since then, the clinical pathogenesis of pheochromocytoma has been known. In the past, pheochromocytoma was considered to be a rare disease. In recent years, the number of cases has increased due to continuous improvement in diagnostic methods. According to statistics, the incidence rate is 0.4 to 2.0% of all cases of hypertension. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: fibromatosis, tuberous sclerosis, hemangioma
Cause
Adrenal medulla hyperfunction
Gene mutation (95%):
The main causes of adrenal medulla hyperfunction are pheochromocytoma, malignant pheochromocytoma and adrenal medulla hyperplasia.
The chromaffin cells are round or elliptical, with a complete envelope, and the surrounding blood vessels are full of anger. The tumor is generally large, with a diameter of 2 to 6 cm, about 90% of which occurs in the adrenal medulla, and the remaining 10% can occur outside the adrenal gland. The site, which occurs outside the adrenal gland, is more common in the retroperitoneal aorta, including chromosome (Zuckerkandl body), but also in the kidney, renal hilum, hilar, pancreatic head, spleen, celiac artery, beside the iliac artery , ovarian, bladder area, pheochromocytoma outside the abdominal cavity is rare, such as the posterior mediastinal spine, occasionally in the neck, intracranial and testicular, adrenal pheochromocytoma 90% is single, bilateral or multiple 10%, the tumor is generally benign (accounting for about 90%), the cut surface is orange, common bleeding, necrosis and cystic changes, blood vessels are rich, interstitial is rare, tumor cells are large, irregular polygons, cytoplasm More granules, similar to normal adrenal medulla cells, but larger, chromate can stain the particles, so called pheochromocytoma, about 10% of pheochromocytoma is malignant, but sometimes from histomorphology Difficult to identify, mainly in its Malignant behavior, i.e. capsular invasion metastasis, lymphatic, liver, bone and lung and other organs.
In a very small number of cases, the clinical manifestations and biochemical tests are consistent with the diagnosis of pheochromocytoma, but no tumor exists, which is caused by adrenal medulla hyperplasia. The proliferating medulla cells are morphologically indistinguishable from normal medulla cells, only the entire adrenal gland is seen. The volume is large, the gland is full, the surface is uplifted, the medulla layer is widened (the ratio to the cortex is greater than 1:10), and the medulla hyperplasia is a bilateral lesion, but the degree of hyperplasia on both sides may be different.
The clinical manifestations are caused by the secretion of a large amount of catecholamines by tumor cells. Adrenal pheochromocytoma mainly secretes epinephrine, while the pheochromocytoma outside the adrenal gland mainly secretes norepinephrine, which is due to the lack of methyl group in ectopic tumor tissue. Due to the transfer of enzymes, the secretion of catecholamines by pheochromocytoma can be intermittent or persistent, resulting in variable clinical symptoms.
Neuroblastoma, ganglioneuroma, can also secrete catecholamines, which have clinical manifestations similar to pheochromocytoma.
Prevention
Adrenal medulla hyperfunction prevention
Adrenal medullary tumors - pheochromocytoma is the main cause of medullary hyperfunction, so there are no effective preventive measures.
Complication
Adrenal medulla hyperfunction Complications fibroids nodular sclerosing hemangioma
Often accompanied by multiple endocrine tumor type II or neuroectodermal dysplasia, such as multiple neurofibromatosis, tuberous sclerosis, multiple trigeminal hemangioma. Can also be complicated by the following diseases, such as heart failure, arrhythmia, hypertensive encephalopathy, lung infections and other abnormal changes. If there is an adrenal medulla hyperthyroidism crisis, blood should be taken immediately to determine urea nitrogen, creatinine, blood sugar, etc., to avoid rolling the tumor site.
Other complications:
1. Cardiovascular complications, catecholaminergic heart disease, arrhythmia, shock.
2. Cerebrovascular complications Stroke, transient ischemic attack (TIA), hypertensive encephalopathy, mental disorders.
3. Others such as diabetes, ischemic enterocolitis, cholelithiasis, etc.
Symptom
Adrenal medulla hyperthyroidism common symptoms polyuria bronchospasm adrenal hyperfunction hyperactivity tachycardia salt corticosteroids excessive blood pressure drop shock
Typical symptoms:
High blood pressure, excessive secretion of catecholamines, abdominal mass.
Examine
Examination of adrenal medulla hyperfunction
1. Adrenal CT scan is preferred. When performing a CT examination, due to changes in body position or injection of venous contrast agents can induce hypertensive seizures, alpha-adrenergic receptor blockers should be used to control hypertension, and phentolamine should be prepared at any time during the scan for urgent needs. .
2. Magnetic resonance imaging (MRI) can show the anatomical relationship and structural characteristics of the tumor and surrounding tissues, and has a high diagnostic value.
3. B is convenient, easy, and low in price, but the sensitivity is not as good as CT and MRI, and it is not easy to find smaller lesions. Can be used as a means of initial screening and positioning.
4.131I-m-iodobenzylamine (MIBG) scintigraphy has a high diagnostic value for the location of extra-adrenal, multiple or malignant metastatic pheochromocytoma lesions, and has qualitative and localized significance, but there are false negatives, so it should be examined The first week was discontinued and the compound iodine was taken to protect the thyroid gland before the test.
5. Adrenal venous intubation blood sampling for plasma CA When the clinical manifestations and biochemical tests support the disease, but the above non-invasive imaging examination failed to locate the tumor, it can be considered. During the operation, it may induce hypertensive crisis, and phentolamine should be prepared for emergency use.
In recent years, 11-carbon hydroxyephedrine, octreotide imaging or PET has been used to diagnose this disease.
Diagnosis
Diagnosis and diagnosis of adrenal medulla hyperfunction
1. Essential hypertension Some patients with essential hypertension exhibit high sympathetic nerve excitability, manifested as palpitations, hyperhidrosis, anxiety, and increased cardiac output. However, the patient's urinary catecholamines are normal. In particular, the determination of catecholamines during the onset of anxiety is more helpful in the exclusion of adrenal medulla.
2. Intracranial disease In the case of intracranial disease combined with high intracranial pressure, symptoms such as severe headaches such as adrenal medulla hyperthyroidism may occur. Patients often have signs of other neurological damage to support the primary disease. However, you should also be alert to adrenal medullary hyperfunction and cerebral hemorrhage.
3. Neuropsychiatric disorders are easily confused with an exacerbation of adrenal medullary dysfunction in anxiety episodes, especially with hyperventilation. But blood pressure is usually normal at an anxiety attack. If blood pressure also rises, it is necessary to measure blood and urine catecholamines to aid identification. Seizures are also similar to adrenal medullary hyperfunction, sometimes blood catecholamines can also be elevated, but urinary catecholamines are normal. There are auras before seizures, abnormal EEG, and effective antiepileptic treatment to help excretion of the adrenal medulla.
4. Menopausal women in the menopausal transition period will have a variety of symptoms caused by estrogen deficiency, such as hot flashes, sweating, irritable, mood swings are difficult to control, similar to adrenal medulla hyperactivity, by understanding menstrual history The determination of sex hormones and CA can be helpful in identification.
5. Other hyperthyroidism presents with high metabolic symptoms, accompanied by high blood pressure. However, the diastolic blood pressure is normal and the catecholamines are not increased. Coronary heart disease angina pectoris, acute myocardial infarction, etc. need to be differentiated from adrenal medullary hyperfunction. Generally, it can be distinguished according to the change of electrocardiogram at the time of onset and the improvement of the treatment of myocardial blood supply. The most critical is the determination of urinary catecholamines.
