An adrenal crisis – is this phaeochromocytoma?

Tanday R, Vakilgilani T, Todd JF, Flora, R and Palazzo, F.

Imperial Centre for Endocrinology, Hammersmith Hospital


We present the case of a 44 year old man who presented with a 10 year history of hypertension, episodic flushing, sweating and bouts of anger.  He has a past medical history of chronic sinusitis and bilateral cataracts.  He is on aspirin and irbesartan. In his family history his father and grandfather died of a myocardial infarction both before the age of 60.  His brother and one of his two sons have hypertension. He works in the removals business.  He is an ex-smoker of 10 pack years and consumes 40 units of alcohol per week. 

He was referred to us from Watford with abnormal urinary biochemistry and a limited MIBG study suggesting phaeochromocytoma.  Repeat biochemistry here demonstrated borderline raised urinary catecholamines and metanephrines at above twice the upper limit of normal, normal 5-HIAA levels and thyroid function.  Our MIBG of the whole body showed uptake in the left adrenal.  In the neuroendocrine MDT it was initially felt that he did not have a phaeochromocytoma as the MIBG uptake was too diffuse and maybe physiological in view of the normal CT component of the study.   He subsequently had a negative clonidine suppression test and his biochemistry partly attributed to obstructive sleep apnoea.   The patient was reassured, continued on doxazosin and kept under surveillance.

Throughout the next 6 months he describe worsening symptoms making  life intolerable.  His hypertension was poorly controlled needing multiple agents and repeat urinary biochemistry showed borderline raised metanephrines and catecholamines.  On repeat imaging at this stage, a MRI adrenal was performed demonstrating a hypervascular 9mm left adrenal lesion.  In the MDT it was now thought that phaeochromocytoma was possible and the decision made for adrenalectomy with the proviso that he may not be cured.  The patient understood this and was keen for surgery.  A left open adrenalectomy was performed and the patient reported sudden reduction in his symptoms with ability to wean his 3 antihypertensives to a single agent.  Urinary biochemistry was now in the lower half of normal range.  The histology however, showed no evidence of a phaeochromocytoma. 

In summary this is an unusual case with biochemical and radiological evidence of a phaeochromocytoma with instant benefit symptomatically and biochemically following adrenalectomy but with normal histology.