Diagnosis and treatment of pheochromocytoma
Endocrinology / / May 11, 2016
Diagnosis and differential diagnosis. diagnosis of pheochromocytoma can present significant challenges.Establishing a correct diagnosis is relatively easy for patients who experience typical crises with increased blood pressure, autonomic and metabolic disorders associated with giperadrenalinemiey, amid normotonii.It facilitates the diagnosis and the presence of a large tumor, accessible to palpation, which occurs in 15% of patients.
Much greater difficulties are encountered in the diagnosis of pheochromocytoma, flowing with constant high blood pressure.About pheochromocytoma should think especially in the presence of hypertension in children and young people who do not have other diseases associated with high blood pressure, such as kidney disease.Sometimes it is possible to establish that in these patients at the beginning there were crises typical of the disease.There may be some value in the presence of pheochromocytoma other family members.
greatest value in such cases is the identification of m
pheochromocytoma, as well as for diagnosis of tumors of the adrenal cortex, adrenal used X-ray region after administration retroperitoneal tissue oxygen or carbon dioxide - oksisuprarenorentgenografiya.The gas in the retroperitoneal space is advantageously introduced through the puncture Presacral.With the introduction of oxygen directly into the perirenal fat describes death by air embolism.Not exclude the possibility of a crisis or a collapse due to irritation of the tumor.
an offer to diagnose pheochromocytomas aortography unsafe.
When extraadrenal location pheochromocytomas X-ray examination often helps to clarify the localization of the tumor, and is a necessary trial laparotomy.
By functional tests used for the diagnosis of pheochromocytomas are samples with adrenoliticheskoe tools and samples, aimed at provocation crisis.
Samples adrenoliticheskoe means used in the presence of patients with resistant hypertension, or during a crisis.For this purpose, there is provided a number of drugs - redzhitin, dibenamin, benzodioxan and domestic product - tropafen.
most reliable results are obtained using redzhitina (phentolamine).The sample is as follows: the patient in a horizontal position establishing a system for drip infusion of 5% glucose solution and re-measured blood pressure before the establishment of the basal pressure.Then, while maintaining the maximum patient rest, through the already-established system 5 mg intravenously per minute redzhitina.After 2 minutes Reduced systolic pressure of not less than 35 mm Hg.Art and diastolic pressure -. no less than 25 mm Hg.Art.He speaks in favor of having pheochromocytoma.Redzhitin can be administered and intramuscularly at the same dose, reduction in blood pressure that occurs when after 20 minutes.
False positive tests with redzhitinom may fall in patients receiving barbiturates, morphine, chloral hydrate, potassium thiocyanate.Lack of response to redzhitin may occur in patients with pheochromocytoma, taking antihypertensive drugs, as well as - in some cases - with long-term course of the disease with persistent hypertension.
Dibenamin administered intravenously at a dose of 7 mg / kg in 300 ml of 5% glucose solution for one hour.This drug is not sufficiently specific - lowers blood pressure not only in patients with pheochromocytoma, but also in hypertension, which makes it less suitable for diagnostic purposes.
Benzodioxan that slowly administered intravenously at a dose of 10 to 15 mg, has a number of side effects (causing shortness of breath, headache, nervousness and fear and so on. D.), Which reduces the value of this drug.
Ppreparat tropafen administered intravenously - 1 ml of 2% solution.It needs to clarify the question of tropafena action specificity, ie its ability to reduce blood pressure in patients with essential hypertension.
In the absence of hypertension in patients can apply the sample, aimed at provocation attack.The simplest of these tests are related to the mechanical stimulation of tumor by palpation or electric current (galvanization) and the cold test (temperature patient hand immersion into water 4 ° C for one minute).Palpation can cause high blood pressure only in the presence of large tumor;cold test is specific enough.
Satisfactory results are obtained by histamine test.Intravenous injection of 0.05 mg of histamine causes a crisis in patients with pheochromocytoma in 2 minutes, provided the cancellation of sedative and narcotic drugs at least two days prior to the study.
certain important test with adrenaline.Patients with pheochromocytoma are less sensitive to adrenaline than healthy people.If react healthy blood pressure rise to the introduction of 0.25 ml of 0.1% epinephrine solution, pheochromocytoma patients to provoke a crisis is necessary to introduce at least 2 ml of the same epinephrine solution.
additional diagnostic value has the ability to stop quickly developing as a result of provocation crisis introduction adrenoliticheskoe funds.
must always be borne in mind that the crisis in patients with pheochromocytoma is very dangerous and can cause serious complications.Therefore, to apply provocative tests can only be in the hospital, being available adrenoliticheskoe means that a crisis can be quickly eliminated.
paramount diagnostic importance is the definition of allocation catecholamines urine.The daily excretion of catecholamine defined fluorometric method in healthy individuals under conditions of rest varies between 15-30 g / 24 h, and 80 to 85% is allocated catecholamines norepinephrine.The voltage conditions and in some diseases, especially when toxic goiter, catecholamine excretion increases, but typically remains significantly lower than in patients with pheochromocytoma.Apparently, the value for the diagnosis of pheochromocytoma a daily excretion of catecholamine not lower than 200 mcg / 24 hours. Isolation of catecholamines in the range of 100 to 200 micrograms / 24 hours does not exclude pheochromocytoma, but it requires repeated measurements, in particular determination of urinary catecholamines duringcrises.
fluorometric method allows to determine separately urinary epinephrine and norepinephrine, and makes it possible to differentiate suspected pheochromocytoma different localization.With a significant increase in the excretion of adrenaline tumors can originate from the adrenal medulla or body Zuckerkandl.In an advantageous increase of noradrenaline possible origin of a tumor of the adrenal glands or vneadrenalovaya its localization.
Rarely described false negative test may depend on the violation of the biosynthesis of hormones in the tumor, reducing kidney function or enhancing catecholamine metabolism in the body.
non-specific fluorescence can cause some drugs - quinidine, tetracycline.
For diagnosis of pheochromocytoma can be used in the determination of urinary catecholamine metabolites - vanilinmindalnoy acid excretion in patients with pheochromocytoma which usually greatly improved.
Due to technical difficulties are less common methods associated with the determination of catecholamines in the blood.
differential diagnosis of pheochromocytoma and hypertensive disease , flowing with crises can present significant challenges.When crises associated with the presence of pheochromocytoma, unlike hypertensive crises, often rises in blood sugar and increased leukocyte count, an increase in temperature is observed.About pheochromocytoma often as possible to think in the presence of hypertension in children and young adults.Suspicion of pheochromocytoma should occur with no effect on the right of the therapy antihypertensives in patients with hypertension.Sometimes, the differential diagnosis is made possible only during the pharmacological tests and, in particular, in determining urinary catecholamines and their metabolites.
Symptomatic hypertension in renal disease rarely occurs with crises.The differential diagnosis is important definition of pathological changes in the urine and detection of impaired kidney function.
mostly on laboratory studies, in particular on the determination of catecholamine excretion in some cases based differential diagnosis of pheochromocytoma and diencephalic crises associated with abnormal hypothalamic region.Diencephalic crises occurring with diverse vegetative disorders and high blood pressure, may be clinically indistinguishable from crises prifeohro-motsitome.Increased excretion of catecholamines at diencephalic crises does not reach the level of their allocation in pheochromocytoma.
Need for differential diagnosis of pheochromocytoma and hyperthyroidism may occur due to the presence in both diseases increased basal metabolic rate and weight loss, as well as due to the fact that many of the symptoms of hyperthyroidism associated with increased tone of the sympathetic division of the autonomic nervous system.For hyperthyroidism are not peculiar to crises with increased blood pressure.Isolation of catecholamines in thyrotoxicosis may be increased due to the excretion of norepinephrine, but does not reach the level inherent in pheochromocytoma patients.Iodine is bound to plasma proteins, and the capture of radioactive iodine by the thyroid gland in patients with pheochromocytoma are usually normal.
In recent years, describes the form of the disease, flowing with hypertensive crisis, not clinically distinguishable from crises in pheochromocytoma, sometimes accompanied by hyperglycemia.In these patients dropped out positive pharmacological tests used for the diagnosis of pheochromocytoma on, and the allocation of catecholamine excretion was at the level corresponding to the lower limits of urinary catecholamines pheochromocytoma patients.One of these patients revealed a tumor of the adrenal cortical layer, after the removal of which the symptoms have disappeared, the other - cystic kidney.It is suggested that these patients had "psevdofeohromotsitomy syndrome" associated with stimulation of the adrenal medulla tumor or cyst kidney.
describes as patients with the syndrome psevdofeohromotsitomy in which the presence of specific crises had hyperplasia of the adrenal medulla.
Pregnant women may experience some difficulties in the differential diagnosis of pheochromocytoma and toxicosis second half of pregnancy.
course and prognosis. Pheochromocytoma has gradually progressive course.Unrecognized timely disease can last for many years.The attacks are becoming more frequent and gradually become more severe, leading to disability of patients.Blood pressure can be a persistent increase.Death may occur during a crisis as a result of a brain hemorrhage, myocardial infarction, acute heart failure.The cause of death can be a vascular collapse under stress.Cases of death due to gastrointestinal bleeding.
With long-term course of the disease with persistent hypertension develop irreversible changes in the kidneys with symptoms of renal failure.
In pregnant women, the presence of pheochromocytoma significantly worsens the prognosis for the mother and fetus.
After surgery to remove the tumor if the patient did not develop irreversible changes due to hypertension, there comes a full recovery.
If symptoms persist or relapse of the disease, one should bear in mind the possibility of multiple tumors.
absolutely unfavorable prognosis When malignant pheochromocytoma.
- Physiology of the adrenal cortex
- diagnosis and course of the syndrome Cushing's
- Treatment syndrome Cushing's
- androgen-producing tumors of the adrenal cortex
- Primary aldosteronism (Conn's syndrome)
- Biological effects of catecholamines
- feminizing tumor of the adrenal cortex
- Diseases of the adrenal medulla
- Treatment of chronic adrenal insufficiency
- congenital (inborn) hyperaldosteronism
- congenital virilizing adrenal hyperplasia
- Treatment of congenital adrenal hyperplasia
- Acute adrenocortical insufficiency
treatment. only radical treatment of pheochromocytoma is surgical removal of the tumor.In the preoperative period is recommended complete rest, the use of sedatives and adrenoliticheskoe funds.Many surgeons recommend redzhitina administration immediately before surgery and during it.
After surgery, there is a risk of collapse may require administration of norepinephrine, and glucocorticoid.The need to address factors that may provoke crises, -.. Excitement, physical stress, heavy meals, etc.
During crises adrenoliticheskoe shows the use of funds - redzhitina (phentolamine) or tropafena.Redzhitin (phentolamine) at the time of crisis is administered intramuscularly or - in case of severe crisis - intravenously (Sol Phentolamini methansulfonici 0,5% -1,0.), Tropafen - IV - 1 ml of 2% solution.
to prevent crises, in case of frequent occurrence, you can assign redzhitin inside (Phentolamini hydrochlorici 0,025- 1-2 tablets in 3-6 hours) or re-enter tropafen.It should be borne in mind the possibility of orthostatic collapse after the introduction adrenoliticheskoe funds.
significantly less effective treatment of crises inhalation of amyl nitrite (3-5 drops), valerian preparations, validol, barbiturates.