Endocrine Abstracts

Phaeochromocytomas are rare catecholamine secreting tumours arising from the chromaffin cells of the adrenal medulla. The annual incidence is approximately 0.8 per 100,000 person-years. Most of them are sporadic but in about 25–30% of patients, they are part of a familial disorder. The classic clinical features are episodic headache, sweating and tachycardia (with or without hypertension). However, a minority of patients are asymptomatic. We present a clinical case in which a patient presented as an acute surgical emergency (posing as a diagnostic and management challenge) subsequently diagnosed as an ‘asymptomatic’ phaeochromocytoma.

A 49 year old lady with sickle cell trait was admitted to the hospital under the care of the surgeons with generalised abdominal pain and vomiting. Investigations showed raised inflammatory markers, anaemia and metabolic acidosis. She was given conservative treatment with fluids and analgesia for a possible diagnosis of sickle cell crisis. She became more unwell with the haemoglobin acutely dropping from 91 to 64 g/L and without any evidence of sickling on the blood film. An urgent abdominal CT scan showed a large left retroperitoneal haemorrhage which was thought to arise from an 8 cm adrenal mass. Following stabilisation with blood transfusion, the surgical team were keen to carry out emergency surgery. However after discussion with the Endocrinologist, who strongly advised against this, surgical treatment was put on hold because of the possibility of the adrenal mass being a phaechromocytoma and the potential risk of a ‘Phaeo crisis’. The patient remained stable and after extensive discussion she was started on alpha blockade with Phenoxybenzamine and transferred to the regional tertiary care centre for elective surgery. Urgent sample for plasma metanephrines was sent which confirmed phaeochromocytoma (Plasma metadrenaline >25,000 - Normal <800). The patient underwent elective adrenalectomy and histology confirmed a benign phaeochromocytoma.

This case highlights the importance of taking a step back in similar situations and thinking about possible phaeochromocytoma even in the absence of typical signs and symptoms.