Phaeochromocytoma is a tumour of the adrenal medullary cells of chromaffin origin, which, although histologically benign, may be dangerous because of excessive secretion of adrenaline and noradrenaline. The growth may not be confined to the adrenals but may occur wherever chromaffin tissue is found, for example along the sympathetic chain, the para-aortic areas, the aortic bifurcation, the retroperitoneum and even in the bladder.Clinical features, resulting from the release of catecholamines, include hypertension, paroxysmal or continuous, hyperhydrosis, hyperglycaemia, increased oxygen consumption, haemoglobin percentage and haematocrit reading, and an elevated basal metabolic rate.The diagnosis is confirmed by measurement of single 24-hour urinary catecholamine or 3-methoxy, 4-hydroxymandelic acid (VMA) values, by certain radiological techniques, and by the effect of certain pharmacological agents, for example histamine to release stored catecholamines and to raise the blood pressure or phentolamine to lower it. Case reportThe patient, a 32-year-old male weighing 79.5 kg, gave a history of low back pain for 2 years and of attacks of sudden uncontrollable aggressive behaviour during the past 18 months with periodic blurred vision.The family history showed that his father, grandfather and great-grandfather had suffered from similar attacks of aggressive behaviour, and a sister had undergone removal of a phaeochromocytoma.Clinical examination revealed a blood pressure of 215/130 mmHg, and a lumbar disc herniation was found at L4-5 level. The attacks of aggressive behaviour were not controlled by drug therapy. Intravenous pyelography showed no abnormality, but on arteriography a large tumour was demonstrated in the right suprarenal area.
Phaeochromocytoma is a tumour of the adrenal medullary cells of chromaffin origin, which, although histologically benign, may be dangerous because of excessive secretion of adrenaline and noradrenaline. The growth may not be confined to the adrenals but may occur wherever chromaffin tissue is found, for example along the sympathetic chain, the para-aortic areas, the aortic bifurcation, the retroperitoneum and even in the bladder.Clinical features, resulting from the release of catecholamines, include hypertension, paroxysmal or continuous, hyperhydrosis, hyperglycaemia, increased oxygen consumption, haemoglobin percentage and haematocrit reading, and an elevated basal metabolic rate.The diagnosis is confirmed by measurement of single 24-hour urinary catecholamine or 3-methoxy, 4-hydroxymandelic acid (VMA) values, by certain radiological techniques, and by the effect of certain pharmacological agents, for example histamine to release stored catecholamines and to raise the blood pressure or phentolamine to lower it. Case reportThe patient, a 32-year-old male weighing 79.5 kg, gave a history of low back pain for 2 years and of attacks of sudden uncontrollable aggressive behaviour during the past 18 months with periodic blurred vision.The family history showed that his father, grandfather and great-grandfather had suffered from similar attacks of aggressive behaviour, and a sister had undergone removal of a phaeochromocytoma.Clinical examination revealed a blood pressure of 215/130 mmHg, and a lumbar disc herniation was found at L4-5 level. The attacks of aggressive behaviour were not controlled by drug therapy. Intravenous pyelography showed no abnormality, but on arteriography a large tumour was demonstrated in the right suprarenal area.
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