Phaeochromocytoma Health Dictionary

Phaeochromocytoma: From 3 Different Sources


A rare tumour of cells that secrete epinephrene (adrenaline) and norepinephrine (noradrenaline). The tumour causes increased production of these hormones, leading to hypertension. The tumours usually develop in the medulla (core) of the adrenal glands, and are most common in young to middle-aged adults.Hypertension is the only sign most of the time, but pressure on the tumour, emotional upset, change in posture, or taking beta-blocker drugs can cause a surge of hormones. This surge brings on a sudden rise in blood pressure, palpitations, headache, nausea, vomiting, facial flushing, sweating, and, sometimes, a feeling of impending death.

Blood tests and urinalysis are used to make a diagnosis.

CT scanning, MRI, and radioisotope scanning may be used to locate the tumours, which are then usually removed surgically.

Follow-up medical checks are required because the condition occasionally recurs.

Health Source: BMA Medical Dictionary
Author: The British Medical Association
A disorder in which a vascular tumour of the adrenal medulla (see ADRENAL GLANDS) develops. The tumour may also affect the structurally similar tissues associated with the chain of sympathetic nerves. There is uncontrolled and irregular secretion of ADRENALINE and NORADRENALINE with the result that the patient suffers from episodes of high blood pressure (HYPERTENSION), raised heart rate, and headache. Surgery to remove the tumour may be possible; if not, drug treatment may help.
Health Source: Medical Dictionary
Author: Health Dictionary
n. a small vascular tumour of the inner region (medulla) of the adrenal gland. Many tumours function by their uncontrolled and irregular secretion of the hormones *adrenaline and *noradrenaline. They cause attacks of raised blood pressure, increased heart rate, palpitations, and headache.
Health Source: Oxford | Concise Colour Medical Dictionary
Author: Jonathan Law, Elizabeth Martin

Hypertension

Means high BLOOD PRESSURE (raised pressure of the circulating blood), but since there is a wide range of ‘normal’ blood pressure in the population, a precise level of pressure above which an individual is deemed hypertensive is arbitrary. (A healthy young adult would be expected to have a systolic pressure of around 120 mm Hg and a diastolic of 80 mm Hg, recorded as 120/80.) Hypertension is not a disease as such but a quantitative deviation from the norm. A person with a pressure higher than the average for his or her age group is usually symptomless – although sometimes such people may develop headaches. The identi?cation of people with hypertension is important because it is a signal that they will be more likely to have a STROKE or myocardial infarct (coronary thrombosis or heart attack) than someone whose pressure is in the ‘normal’ range. Preventive steps can then be taken to lessen the likelihood of their developing these potentially life-threatening conditions.

Blood pressure is measured using two values. The systolic pressure – the greater of the two – represents the pressure when blood is pumped from the left VENTRICLE of the heart into the AORTA. The diastolic pressure is the measurement when both ventricles relax between beats. The pressures are measured in millimetres (mm) of mercury (Hg). Despite the grey area between normal and raised blood pressure, the World Health Organisation (WHO) has de?ned hypertension as a blood pressure consistently greater than 160 mm Hg (systolic) and 95 mm Hg (diastolic). Young children have readings well below these, but blood pressure rises with age and a healthy person may well live symptom free with a systolic pressure above the WHO ?gure. A useful working de?nition of hypertension is the ?gure at which the bene?ts of treating the condition outweigh the risks and costs of the treatment.

Between 10 and 20 per cent of the adult population in the UK has hypertension, with more men than women affected. Incidence is highest in the middle-aged and elderly. Because most people with hypertension are symptomless, the condition is often ?rst identi?ed during a routine medical examination, otherwise a diagnosis is usually made when complications occur. Many people’s blood pressure rises when they are anxious or after exercise, so if someone’s pressure is above normal at the ?rst testing, it should be taken again after, say, 10 minutes’ rest, by which time the reading should have settled to the person’s regular level. BP measurements should then be taken on two subsequent occasions. If the pressure is still high, the cause needs to be determined: this is done using a combination of personal and family histories (hypertension can run in families), a physical examination and investigations, including an ECG and blood tests for renal disease.

Over 90 per cent of hypertensive people have no immediately identi?able cause for their condition. They are described as having essential hypertension. In those patients with an identi?able cause, the hypertension is described as secondary. Among the causes of secondary hypertension are:

Lifestyle factors such as smoking, alcohol, stress, excessive dietary salt and obesity.

Diseases of the KIDNEYS.

Pregnancy (ECLAMPSIA).

Various ENDOCRINE disorders – for example, PHAEOCHROMOCYTOMA, CUSHING’S DISEASE, ACROMEGALY, thyrotoxicosis (see under THYROID GLAND, DISEASES OF).

COARCTATION OF THE AORTA.

Drugs – for example, oestrogen-containing oral contraceptives (see under CONTRACEPTION), ANABOLIC STEROIDS, CORTICOSTEROIDS, NON-STEROIDAL ANTIINFLAMMATORY DRUGS (NSAIDS).

Treatment People with severe hypertension may need prompt admission to hospital for urgent investigation and treatment. Those with a mild to moderate rise in blood pressure for which no cause is identi?able should be advised to change their lifestyle: smokers should stop the habit, and those with high alcohol consumption should greatly reduce or stop their drinking. Obese people should reduce their food consumption, especially of animal fats, and take more exercise. Everyone with hypertension should follow a low-salt diet and take regular exercise. Patients should also be taught how to relax, which helps to reduce blood pressure and, if they have a stressful life, working patterns should be modi?ed if possible. If these lifestyle changes do not reduce a person’s blood pressure su?ciently, drugs to achieve this will be needed. A wide range of anti-hypertensive drugs are available on prescription.

A ?rst-line treatment is one of the THIAZIDES, e?ective at a low dosage and especially useful in the elderly. Beta blockers (see BETAADRENOCEPTOR-BLOCKING DRUGS), such as oxprenolol, acebutol or atenolol, are also ?rst-line treatments. ACE inhibitors (see ANGIOTENSIN-CONVERTING ENZYME (ACE) INHIBITORS) and CALCIUM-CHANNEL BLOCKERS can be used if the ?rst-line choices are not e?ective. The drug treatment of hypertension is complex, and sometimes various drugs or combinations of drugs have to be tried to ?nd what regimen is e?ective and suits the patient. Mild to moderate hypertension can usually be treated in general practice, but patients who do not respond or have complications will normally require specialist advice. Patients on anti-hypertensive treatments require regular monitoring, and, as treatment may be necessary for several years, particular attention should be paid to identifying sideeffects. Nevertheless, e?ective treatment of hypertension does enable affected individuals to live longer and more comfortable lives than would otherwise be the case. Older people with moderately raised blood pressure are often able to live with the condition, and treatment with anti-hypertensive drugs may produce symptoms of HYPOTENSION.

In summary, hypertension is a complex disorder, with di?erent patients responding di?erently to treatment. So the condition sometimes requires careful assessment before the most e?ective therapy for a particular individual is identi?ed, and continued monitoring of patients with the disorder is advisable.

Complications Untreated hypertension may eventually result in serious complications. People with high blood pressure have blood vessels with thickened, less ?exible walls, a narrowed LUMEN and convoluted shape. Sometimes arteries become rigid. ANEURYSM may develop and widespread ATHEROMA (fat deposits) is apparent in the arterial linings. Such changes adversely affect the blood supply to body tissues and organs and so damage their functioning. Patients suffer STROKE (haemorrhage from or thrombosis in the arteries of the BRAIN) and heart attacks (coronary thrombosis

– see HEART, DISEASES OF). Those with hypertension may suffer damage to the retina of the EYE and to the OPTIC DISC. Indeed, the diagnosis of hypertension is sometimes made during a routine eye test, when the doctor or optician notices changes in the retinal arteries or optic disc. Kidney function is often affected, with patients excreting protein and excessive salt in their urine. Occasionally someone with persistent hypertension may suffer an acceleration of damage to the blood vessels – a condition described as ‘malignant’ hypertension, and one requiring urgent hospital treatment.

Hypertension is a potentially dangerous disease because it develops into a cycle of self-perpetuating damage. Faulty blood vessels lead to high blood pressure which in turn aggravates the damage in the vessels and thus in the tissues and organs they supply with blood; this further raises the affected individual’s blood pressure and the pathological cycle continues.... hypertension

Phenoxybenzamine

An alpha-adrenoceptor blocking drug (see ADRENERGIC RECEPTORS) used in the treatment of HYPERTENSION caused by PHAEOCHROMOCYTOMA.... phenoxybenzamine

Adrenal Tumours

Cancerous or noncancerous tumours in the adrenal glands, usually causing excess secretion of hormones. Adrenal tumours are rare. Tumours of the adrenal cortex may secrete aldosterone, causing primary aldosteronism, or hydrocortisone, causing Cushing’s syndrome. Tumours of the medulla may cause excess secretion of adrenaline and noradrenaline. Two types of tumour affect the medulla: phaeochromocytoma and neuroblastoma, which affects children. These tumours cause intermittent hypertension and sweating attacks. Surgical removal of a tumour usually cures these conditions.... adrenal tumours

Apud Cell Tumour

A growth composed of cells that produce various hormones. These cells, amine precursor uptake and decarboxylation (APUD) cells, occur in different parts of the body. Some tumours of the thyroid gland, pancreas, and lungs are cell tumours, as are a carcinoid tumour (see carcinoid syndrome) and phaeochromocytoma (a type of adrenal tumour).... apud cell tumour

Catecholamines

pl. n. a group of physiologically important substances, including *adrenaline, *noradrenaline, and *dopamine, having various different roles (mainly as *neurotransmitters) in the functioning of the sympathetic and central nervous systems. Chemically, all contain a benzene ring with adjacent hydroxyl groups (catechol) and an amine group on a side chain. Catecholamines act at both alpha (?) and beta (?) *adrenoceptors. Some tumours (e.g. *phaeochromocytoma) secrete excess catecholamines.... catecholamines

Clonidine

n. a drug that interacts with *adrenoceptors in the brain to reduce sympathetic stimulation of the arteries (see catecholamines; noradrenaline). It is occasionally used to treat high blood pressure Common side-effects include drowsiness and dry mouth, and sudden withdrawal may worsen the hypertension.

In the clonidine suppression test, used in the diagnosis of *phaeochromocytoma, clonidine is administered orally and plasma catecholamines sampled then and at hourly intervals for three hours. In normal subjects clonidine suppresses release of catecholamines and plasma levels are reduced, but in people with phaeochromocytoma plasma catecholamines remain elevated.... clonidine

Glucagon Stimulation Test

a test for *phaeochromocytomas not displaying typically high levels of plasma *catecholamines. An intravenous bolus of *glucagon is administered and the test is positive when there is a threefold increase in plasma catecholamine levels with a consequent rise in blood pressure. The test is now very rarely used due to safer and more sensitive screening tests, combined with modern tumour imaging techniques.... glucagon stimulation test

Haemangioblastoma

(Lindau’s tumour) n. a tumour of the brain or spinal cord arising in the blood vessels of the meninges or brain. It is often associated with *phaeochromocytoma and *syringomyelia. See also von Hippel-Lindau disease.... haemangioblastoma

Mens

multiple endocrine neoplasia syndromes, designated as type 1 (Wermer’s syndrome), type 2A (Sipple’s syndrome), and type 2B. These involve tumour formation or hyperplasia in various combinations of endocrine glands. Type 1 involves the parathyroid, pituitary, and pancreas, whereas type 2A involves the thyroid medullary cells, the adrenal medulla (*phaeochromocytoma), and the parathyroids. Type 2B is similar to 2A, but patients tend to resemble people with *Marfan’s syndrome and have multiple *neuromas on their mucous membranes. These conditions are inherited as autosomal *dominant characteristics.... mens

Metanephrine And Normetanephrine

metabolites of the hormones adrenaline and noradrenaline, respectively. Both hormones are released in excess from a *phaeochromocytoma, and measurement of their metabolites is the most reliable screening test for this rare but potentially lethal condition. The test is best carried out on a complete 24-hour urine collection.... metanephrine and normetanephrine

Mibg

Meta-IodoBenzylGuanidine: a radioactive *tracer, labelled with iodine-123 or iodine-131, which binds to adrenergic nerve tissue. With the aid of a gamma camera, it can be used to detect the presence of a range of adrenergic tumours, including *neuroblastoma and *phaeochromocytoma.... mibg

Paraganglioma

n. a tumour, related to *phaeochromocytoma, arising from *paraganglion cells. Such tumours can occur around the aorta, the carotid artery (carotid body tumour), and the cervical portion of the vagus nerve (*glomus tumour), as well as in the abdomen and the eye. They are usually benign and treated by surgery, but occasionally can be malignant, requiring systemic treatment.... paraganglioma

Phentolamine

n. a drug that dilates blood vessels (see alpha blocker) and is used to reduce high blood pressure in patients with *phaeochromocytoma. Side-effects include fast heartbeat and digestive upsets.... phentolamine

Vanillylmandelic Acid

(VMA) a metabolite of *catecholamines excreted in abnormal amounts in the urine in conditions of excess catecholamine production, such as *phaeochromocytoma. The measurement of VMA levels in a 24-hour urine sample was formerly used as a screening test for this condition, but in the UK it has been largely superseded by the urinary metanephrine test (see metanephrine and normetanephrine).... vanillylmandelic acid



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