Joyce SY Yau, June KY Li, Vicki HK Tam, LM Fung, CK Yeung, KW Chan, KM Lee, KF Lee, WS Cheung, Vincent TF Yeung, YP Yuen, WK Kwan. Department of Medicine, Yan Chai Hospital, Hong Kong
OBJECTIVE. To review the clinical manifestations of phaeochromocytoma in a Hong Kong Chinese population.
DESIGN. Retrospective review.
SETTING. Five public hospitals in Hong Kong.
PATIENTS. Seventeen patients with operated phaeochromocytoma between 1994 and 2003 were reviewed retrospectively.
RESULTS. Six patients (35%) were men, 11 (65%) were women. The mean age at presentation was 47 (range, 17-72) years. The diagnosis post-presentation was delayed by 1 to 132 months. Over 70% of the patients had hypertension. The most frequent symptoms were headache (53%), palpitations (53%), and sweating (41%); all these symptoms were present in 24% of the patients. Four (24%) had hereditary phaeochromocytoma/paraganglioma syndrome. The sensitivity of 24-hour urinary catecholamine measurements was 82%. Mean urinary adrenaline and noradrenaline concentrations were respectively 7- and 8-fold greater than the upper reference limits. Computed tomography and metaiodobenzylguanidine scintigraphy were the most widely used means for tumour localisation (sensitivity, 100% and 87% respectively). Approximately 65% of the patients had intra-adrenal tumours; 53% were on right side, 18% were bilateral. All the patients were prescribed phenoxybenzamine (dosage range, 20-120 mg/day) preoperatively. Two thirds of the patients had improved blood pressure 1 year after the operation. No malignancy was reported after a mean follow-up period of 7 years.
CONCLUSION. Our series of patients with phaeochromocytomas commonly had a high frequency of normotension and extra-adrenal tumours. A high index of clinical suspicion and appropriate biochemical investigations are necessary to make the diagnosis, especially for patients manifesting adrenal incidentaloma and extra-adrenal lesion.
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