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The perioperative management of a 39-year-old Paragangllomas arise from a widely disseminated system of small sensory and neurosecretory organs that develops in fetal life and subsequently decreases in size. t Phaeochromocytomas are classical examples of endocrinologically active chromaffin paragangliomas. Typical nonchromaffin paragangliomas (NCPG) are tumours of the carotid body and glomus jugulare. A NCPG is usually eudocrinologically inactive, but there are rare cases, such as the one described in this report, of a NCPG producing and releasing catecholamines. 2'3 Urinary bladder paragangliomas are uncommon,I have a familial tendency 4 and a mean age of presentation of 38 years. When these paragangliomas stain chromaffinpositive and are endocrinologically active, they have been called bladder phaeochromocytomas. Patients with these "phaeochromocytomas" may present with a classical triad s of haematuria, hypertension and micturitional attacks typical of a "phaeoehromocytoma syndrome ''6 (headaches, tremulousness, anxiousness and a pounding sensation). Fortunately, bladder phaeochromocytomas are rare, representing only 0.06 per cent of bladder tumours. 2 Seven to 20 per cent of these tumours are malignant 1,5 and can be detected by increased serum dopa and dopamine levels]In this report, we describe the anaesthetic management of a patient who underwent incomplete resection of a malignant, highly functional NCPG of the bladder. This tumour, which to the best of our knowledge has never been reported before, is difficult to distinguish from a phaeochromocytoma. The case was complicated by atypical presentation, substantial release of catecholamines from the tumour, difficult preoperative control of hypertension and complex surgical resection. Case reportA 39-year-old, 61 kg, white male was admitted to the urology service with weight loss, nausea, vomiting, malaise, headaches, hypertension, bladder mass, right flank pain and right hydronephrosis. On examination he was eachectic, hypertensive (BP 250/140 mmHg), CAN J ANAESTH 1989 / 36:2 / pp215-8
The perioperative management of a 39-year-old Paragangllomas arise from a widely disseminated system of small sensory and neurosecretory organs that develops in fetal life and subsequently decreases in size. t Phaeochromocytomas are classical examples of endocrinologically active chromaffin paragangliomas. Typical nonchromaffin paragangliomas (NCPG) are tumours of the carotid body and glomus jugulare. A NCPG is usually eudocrinologically inactive, but there are rare cases, such as the one described in this report, of a NCPG producing and releasing catecholamines. 2'3 Urinary bladder paragangliomas are uncommon,I have a familial tendency 4 and a mean age of presentation of 38 years. When these paragangliomas stain chromaffinpositive and are endocrinologically active, they have been called bladder phaeochromocytomas. Patients with these "phaeochromocytomas" may present with a classical triad s of haematuria, hypertension and micturitional attacks typical of a "phaeoehromocytoma syndrome ''6 (headaches, tremulousness, anxiousness and a pounding sensation). Fortunately, bladder phaeochromocytomas are rare, representing only 0.06 per cent of bladder tumours. 2 Seven to 20 per cent of these tumours are malignant 1,5 and can be detected by increased serum dopa and dopamine levels]In this report, we describe the anaesthetic management of a patient who underwent incomplete resection of a malignant, highly functional NCPG of the bladder. This tumour, which to the best of our knowledge has never been reported before, is difficult to distinguish from a phaeochromocytoma. The case was complicated by atypical presentation, substantial release of catecholamines from the tumour, difficult preoperative control of hypertension and complex surgical resection. Case reportA 39-year-old, 61 kg, white male was admitted to the urology service with weight loss, nausea, vomiting, malaise, headaches, hypertension, bladder mass, right flank pain and right hydronephrosis. On examination he was eachectic, hypertensive (BP 250/140 mmHg), CAN J ANAESTH 1989 / 36:2 / pp215-8
Pheochromocytoma (Pheo) is an uncommon neoplasm producing blood pressure troubles and it may be undiagnosed in chronic dialyzed patients in whom hypertension is a common finding. The symptoms in Pheo syndrome depends on the prevalent catecholamine released, the most common being epinephrine (E) and norepinephrine (NE). Recently, a particular clinical picture has been described for dopamine (DA)-producing Pheos, in whom a normo-hypotensive status is more often observed. The authors report a case of mainly dopamine-producing Pheo in a long-term dialyzed patient, successfully treated with adrenalectomy. The main steps in diagnosis and preoperative management are described and debated also in view of the particular background produced by the end-stage renal failure. The common imaging techniques adopted for adrenal medullary neoplasms (US, CT, MIBG scintiscan) confirmed to be decisive for diagnosis; HPLC assay of plasma catecholamines is the only biochemical test available in these patients although its significance is questionable due to the poor knowledge of catecholamine metabolism in chronic renal failure. The clinical findings observed in this case seem in disagreement with those already reported in DA producing Pheos. Pheo in hemodialyzed patients is a rare event and it may be hidden by other more common causes of hypertension. However, more awareness from the medical staff allows to diagnose the neoplasm correctly by the currently available methods and to plan a safe surgical therapy also in high-risk patients.
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