Endocrine Abstracts

A 21-year-old female patient was referred from the eye clinic after she was noted to have bilateral papilloedema during a routine eye examination. She gave a 3 months history of intermittent headaches and fleeting episodes of profuse sweating and rash, unrelated to the headaches. She had been investigated by the GP with routine blood tests which were found to be normal.

On examination, she was noted to be tachycardic at 100 beats/min and hypertensive at 189/130 mmHg. She had erythematous maculopapular rashes over the lower forearm and hand bilaterally. Examination of the fundus confirmed bilateral papilloedema. ECG showed evidence of left axis deviation and left ventricular hypertrophy. CXR showed normal sized heart and clear lung fields. She was commenced on Nifedipine MR 20 mg bd. Ultrasound of the abdomen revealed a 4 cm right adrenal mass. MRI of the brain was reported as normal. An MRI of the adrenals and MIBG scan were arranged.

Nifedipine was stopped and she was commenced on Phenoxybenzamine 10 mg bd. As the patient remained well with improved control of her blood pressure, she was commenced on Propranolol 40 mg bd after 3 days.

The patient unfortunately had a cardiac arrest and died despite prolonged resuscitation. The coroner reported cause of death as acute pulmonary oedema secondary to malignant hypertension due to a right adrenal phaeochromocytoma.

Results of 3×24 h urine catecholamines obtained thereafter showed noradrenaline levels almost 100 times more than the normal levels at 11 800/24 700/19 100 μg/24 h (normal range <100) and normetanephrine levels at 100/47/30 μg/24 h (normal range <3.3).

This case highlights the difficulty in treating these extremely rare tumours and the potentially fatal complications due to an adrenergic crises in these patients.