Presents with palpitations, diaphoresis, pallor, and paroxysmal hypertension.
Risk factors include multiple endocrine neoplasia type 2, Von Hippel-Lindau syndrome, and neurofibromatosis type 1.
Diagnosed by increased levels of urine and serum catecholamines, metanephrines, and normetanephrines.
Treatment includes medical therapy for hypertension (phenoxybenzamine, phentolamine, alpha-blockers) and surgical excision of tumour (open or laparoscopic adrenalectomy). For unresectable tumours, alternative approaches include chemotherapy or radiopharmaceuticals.
Complications include hypertensive crisis, myocardial infarction, and hypotension.
A tumour arising from catecholamine-producing chromaffin cells of the adrenal medulla that classically presents with headaches, diaphoresis, and palpitations in the setting of paroxysmal hypertension. Symptoms are usually episodic and tend to progress as the tumour grows. The majority of these tumours are benign. About 80% to 90% arise in the adrenal gland; the remainder being extra-adrenal in origin and most commonly found in the head and neck. These tumours mostly develop sporadically. Up to 40% of cases are a manifestation of a hereditary syndrome, such as multiple endocrine neoplasia type 2 or Von Hippel-Lindau syndrome. The tumour was named phaeochromocytoma, after its characteristic 'dusky-coloured tumour' appearance, by Pick in 1912.
History and exam
- presence of risk factors
- hypertensive retinopathy
- impaired glucose tolerance/diabetes mellitus
- family history of endocrine disorders
- history of prior phaeochromocytoma
- tachyarrhythmias and myocardial infarction
- panic attacks or a 'sense of doom'
- serum calcium
- serum potassium
- chromogranin A
- clonidine suppression test
- MRI of the abdomen and pelvis
- CT scan of the abdomen and pelvis
- I-123 metaiodobenzylguanidine (MIBG) scintigraphy
- 18F-fluoro-2 deoxy-D-glucose (18F-FDG) positron emission tomography (PET)
Associate Professor of Medicine
Medical College of Georgia
BS declares that she has no competing interests.
Dr Bridget Sinnott would like to gratefully acknowledge Dr Sidhbh Gallagher, a previous contributor to this topic. SG declares that she has no competing interests.
Clinical Endocrinologist and Research Scientist
Department of Endocrinology, Diabetes, and Metabolism
BAH declares that she has no competing interests.
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