Practice Easily Missed?

Phaeochromocytoma

BMJ 2012; 344 doi: http://dx.doi.org/10.1136/bmj.e1042 (Published 20 February 2012) Cite this as: BMJ 2012;344:e1042
  1. Angus G Jones, specialist registrar1,
  2. Philip H Evans, general practitioner2, senior clinical research fellow3,
  3. Bijay Vaidya, consultant endocrinologist and honorary reader13
  1. 1Department of Endocrinology, Royal Devon and Exeter Hospital, Exeter EX2 5DW, UK
  2. 2St Leonard’s Practice, Exeter
  3. 3Peninsula Medical School, Exeter
  1. Correspondence to: B Vaidya bijay.vaidya{at}pms.ac.uk
  • Accepted 20 December 2011

A 51 year old woman presented with a few months’ history of episodic anxiety associated with sweating, flushing, and palpitations. The symptoms were thought to be menopausal but they were not relieved by oestrogen hormone replacement therapy. She also had a history of hypertension that was increasingly difficult to control, despite treatment with three antihypertensive agents. Her general practitioner, concerned about a possible phaeochromocytoma, referred her to an endocrinologist. Further investigation showed markedly raised urine metanephrines and a right adrenal mass on computed tomography, consistent with a diagnosis of right adrenal phaeochromocytoma.

What is a phaeochromocytoma?

A phaeochromocytoma is a catecholamine producing tumour arising from the chromaffin cells of the adrenal medulla (85% of cases) or extra-adrenal paraganglia.1 Although it may be malignant, symptoms usually result from the excess secretion of catecholamines.

Why is phaeochromocytoma missed?

Phaeochromocytomas are uncommon in secondary care and rare in primary care. The symptoms and signs of a phaeochromocytoma, which vary greatly, are often non-specific and similar to those of many common conditions, such as anxiety. The so called classic symptoms of a phaeochromocytoma, such as paroxysmal headache, palpitations, and sweating, are often absent. Hypertension may not be present in 10-20% of cases.1 As a result, diagnosis is often delayed, with an average interval of three years between the initial symptoms and diagnosis, and many cases are diagnosed only at autopsy.2 3 4

How common is phaeochromocytoma?

  • The prevalence of phaeochromocytoma in hypertensive patients attending general medical outpatient clinics is 0.1-0.6%1

  • Autopsy studies have shown a prevalence of 0.05%, but many of these cases had not been diagnosed in life3

Why does this matter?

Although we estimate from prevalence data3 that an average full time general practitioner will have only about …

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