Q J Med 1997; 90: 51-60
© 1997 Association of Physicians
Phaeochromocytoma: a ten-year survey
From the Division of Endocrinology & Metabolism, Department of Medicine, Dalhousie University Halifax, Nova Scotia, Canada
Address correspondence to Dr K.-C. Loh, Metabolic Research Unit, University of California San Francisco, Box 0540, HSW 1146, 513 Parnassus Avenue, San Francisco, CA 94143–0540, USA
Received 15 April 1996 and in revised form 31 October 1996
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Abstract |
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We retrospectively evaluated our experience with phaeochromocytoma from January 1986 to December 1995. There were 18 patients with surgically-proven phaeochromocytoma: three males, 15 females, aged 12–81 years (mean 42 years) at diagnosis. Sixteen were hypertensive; only 6/18 presented with two or more of the classical triad of headaches, palpitations and diaphoresis. One patient presented with hypertensive crisis. Duration of symptoms prior to diagnosis was 2 weeks to 6 years, mean 16.4 months. Sixteen patients had adrenal tumours and two had extra-adrenal tumours or para-gangliomas. One had bilateral adrenal tumours and two had a combination of both adrenal and extra-adrenal tumours. There were four familial cases: two had multiple endocrine neoplasia type IIA (MEN-IIA), one had neurofibromatosis type I (NF-I) and one von Hippel-Lindau (VHL) disease. One patient had Cushing's syndrome arising from ectopic production of adrenocorticotropic hormone (ACTH) by the phaeochromocytoma. Disease was recurrent in three patients. Pre-operative diagnosis was confirmed mainly by elevated urine vanillylmandelic acid (VMA) and/or catecholamine levels. Twelve patients had plasma catecholamine determinations: noradrenaline was elevated in all, adrenaline in six and dopamine in two. Pre-operative localization was by CT scan or MR imaging in all patients. At follow-up of 1–10 years (median 4.8 years), 15 patients were cured surgically while two were asymptomatic despite recurrence of disease. One patient with recurrent paragangliomas died post-operatively.
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