Pheochromocytoma: evaluation, diagnosis, and treatment.
Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system. Signs and symptoms are generally related to catecholamine excess; these include hypertension, sweating, palpitatione, headaches, and anxiety attacks. Abdominal imaging and 24-h urine collection for catecholamines are usually be sufficient for diagnosis. Catecholamine blockade with phenoxybenzamine and metyrosine generally ameliorates symptoms and is necessary to prevent hypertensive crisis during surgery. Standard treatment is laparoscopic adrenalectomy, although partial adrenalectomy is gaining enthusiastic support in familial forms of pheochromocytoma. Pheochromocytomas have been estimated to be present in approximately 0.3% of patients undergoing evaluation for secondary causes of hypertension [41]. Pheochromocytomas are usually curable if diagnosed and treated properly, but they can be fatal if they are not diagnosed or are managed inappropriately. Autopsy series suggest that many pheochromocytomas are not clinically suspected and that the undiagnosed tumor can be associated with morbid consequences [42].
Walther
M M
MM
Urologic Oncology Branch, DCS/NCI/NIH, Bethesda, MD 20892-1501, USA. macw@nih.gov
Keiser
H R
HR
Linehan
W M
WM
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000601:surgery* / Q000652:urine; D000315:Adrenalectomy / Q000379:methods; D002395:Catecholamines / Q000652:urine; D003937:Diagnosis, Differential; D006801:Humans; D010535:Laparoscopy; D008279:Magnetic Resonance Imaging; D009364:Neoplasm Recurrence, Local; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000601:surgery* / Q000652:urine; D011379:Prognosis; D014057:Tomography, X-Ray Computed
D002395:Catecholamines