Phaeochromocytoma: a ten-year survey.
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 paragangliomas. 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.
Loh
K C
KC
Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.
Shlossberg
A H
AH
Abbott
E C
EC
Salisbury
S R
SR
Tan
M H
MH
D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy*; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001145:Arrhythmias, Cardiac / Q000150:complications; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006261:Headache / Q000150:complications; D006801:Humans; D006973:Hypertension / Q000150:complications; D008297:Male; D008875:Middle Aged; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D016896:Treatment Outcome; D016482:Urinalysis