Maria Silveira, Dorset County Hospital
The clinical presentations of the patient with pheochromocytoma -- a rare endocrine neoplasm -- include adrenal incidentaloma, hypertensive paroxysms, sustained apparent polygenic hypertension, hypertension in pregnancy, and hypertensive crisis induced by anaesthesia. Although when undiagnosed a pheochromocytoma can be lethal, it can usually be cured with surgery. Biochemical documentation with measurements of fractionated metanephrines and catecholamines should precede imaging studies. Abdomen and pelvis computed imaging is usually the first imaging test. Careful preoperative pharmacologic preparation is important for a successful surgical outcome. Adrenal pheochromocytomas can usually be removed laparoscopically, whereas, catecholamine-secreting paragangliomas typically require an open approach. All first degree relatives of pheochromocytoma patients should have biochemical testing. In addition, molecular genetic testing for germline mutations should be considered in most patients with adrenal pheochromocytoma and in all patients with paraganglioma.
I present a case of a 73 year old male with a background of HT, IHD, RA, IBD, Type 2 DM who while being investigated for iron deficiency anaemia, CT abdomen showed an incidental finding of 5cm adrenal mass, no symptoms of phaeochromocytoma
Investigations showed an elevated 24 hour metanephrines 1603 and normetanephrine 4423, 24 hour urine cortisol was normal.
Overnight dexamethasone and low dose dexamethasone suppression test did not suppress the cortisol suspecting that it is due to high CBG levels rather than co-existing Cushing’s disease.
Hypertension was treated with doxazosin initially with1 mg and gradually titrated to 16mg and bisoprolol10mg.
Laporoscopic surgery was performed and histology confirmed pheochromocytoma, there were some mitotic figures but no other malignant features. He had an excellent recovery, doxasosin was stopped and echocardiogram showed good LV function and dilated left atrium. Referred to genetist to discuss susceptibility genes and also to factor in what his histology means in terms of angiomyolipoma in his daughter.Point of testing here would be to ensure that we are not dealing with VHL