PHEOCHROMOCYTOMA - A 10-YEAR SURVEY

We retrospectively evaluated our experience with phaeochromocytoma fro m January 1986 to December 1995. There were 18 patients with surgicall y-proven phaeochromocytoma: three males, 15 females, aged 12-81 years (mean 42 years) at diagnosis. Sixteen were hypertensive; only 6/18 pre sented with two or more of the classical triad of headaches, palpitati ons and diaphoresis. One patient presented with hypertensive crisis. D uration of symptoms prior to diagnosis was 2 weeks to 6 years, mean 16 .4 months. Sixteen patients had adrenal tumours and two had extra-adre nal tumours or paragangliomas. One had bilateral adrenal tumours and t wo had a combination of both adrenal and extraadrenal tumours. There w ere 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 Gushing's syndrome arising from ectopic production of adrenocorticotropic hormone (ACTH) by the phaeo chromocytoma. Disease was recurrent in three patients. Pre-operative d iagnosis was confirmed mainly by elevated urine vanillylmandelic acid (VMA) and/or catecholamine levels. Twelve patients had plasma catechol amine 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 followup of 1-10 years (median 4.8 yea rs), 15 patients were cured surgically while two were asymptomatic des pite recurrence of disease. One patient with recurrent paragangliomas died post-operatively.