Increased use of abdominal imaging has resulted in vast numbers of adrenal incidentalomas being identified (Bovio et al., 2006). Guidelines exist for identifying hormone excess and diagnosing malignancy but there is little evidence about how to manage phaeochromocytoma in patients who are poor surgical candidates. We present the case of a frail 88 year-old lady who was found to have a right adrenal incidentaloma during assessment for abdominal pain. Mrs R is able to mobilise short distances with a frame. She has a history of falls associated with a postural hypotension, hypertension, previous transient ischaemic attacks, diverticular disease, fractures and a mastectomy for breast cancer. She was found to have a 28 mm right adrenal mass on CT-abdomen. Biochemical work-up showed significantly raised plasma metanephrines. A repeat supine resting sample confirmed a raised plasma normetanephrine at 2678 pmol/l (<720 pmol/l) and raised plasma metanephrines at 984 pmol/l (<450 pmol/l). Other biochemistry was normal. In Endocrinology clinic she described several years of symptoms, including a peculiar feeling in her head, a recurrent sensation of doom and intermittent palpitations. She was also found to be significantly hypertensive. She was not felt to be a surgical candidate in light of frailty and co-morbidities, and was equally not keen on embarking on an operation. The decision was made to manage her phaeochromocytoma conservatively. Doxazocin was chosen, due to its lesser side-effect profile compared to phenoxybenzamine. It was started at a low dose and slowly titrated, with weekly assessment in the Day Unit. Her blood pressure slowly improved but controlling her symptoms remains problematic. With gradual titration and encouraging good salt and water intake, postural symptoms have been minimal and she has not experienced falls. This case illustrates a new population in which phaeochromocytoma diagnoses are being made. Little data is available regarding prognosis when such conditions are managed conservatively long-term, although some comparisons may be possible from looking at patients with metastatic phaeochromocytoma and familial paraganglioma syndromes in whom surgery is no longer feasible. Alpha-blockade is often poorly tolerated and it remains to see if we will achieve adequate blood pressure control without significant side-effects. In this case, we are balancing the risks of adrenalectomy against the risks of ongoing normetadrenaline excess. We suggest there is a need to develop a conservative care pathway to help appropriately manage these patients, balancing quality of life with the burden of symptoms and medications, and collect data on outcomes.
16 - 18 Apr 2018
Society for Endocrinology