Adrenal phaeochromocytoma in familial neurofibromatosis with initial control of hypertension by labetalol

Abstract

Neurofibromatosis is an autosomal dominant ectodermal dysplasia with a frequency of 1 in 2500. These patients have a considerably increased chance of malignancy, mainly due to an excess incidence of specific neural tumours. A case is described with a strong family history of malignancy in whom paroxysmal hypertension due to an adrenal phaeochromocytoma was satisfactorily controlled with oral labetalol. The tumour was successfully located preoperatively by intravenous urography, selective venous sampling for plasma noradrenaline and arteriography.