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 tumor (open or laparoscopic adrenalectomy). For unresectable tumors, alternative approaches include chemotherapy or radiopharmaceuticals.
Complications include hypertensive crisis, myocardial infarction, and hypotension.
A tumor 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 tumor grows. The majority of these tumors 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 tumors 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 tumor was named pheochromocytoma, after its characteristic "dusky-colored tumor" appearance, by Pick in 1912.
History and exam
Key diagnostic factors
- hypertensive retinopathy
- impaired glucose tolerance/diabetes mellitus
- family history of endocrine disorders
- history of prior pheochromocytoma
- tachyarrhythmias and myocardial infarction
- panic attacks or a "sense of doom
Other diagnostic factors
- orthostatic hypotension
- Cushing syndrome
- abdominal masses
- Multiple endocrine neoplasia (MEN) syndrome type 2A and B
- Von Hippel-Lindau (VHL) disease
- neurofibromatosis type 1 (NF1)
- succinate dehydrogenase (SDH) subunit B, C, and D gene mutations
1st investigations to order
- 24-hour urine collection for catecholamines, metanephrines, normetanephrines, and creatinine
- serum free metanephrines and normetanephrines
- plasma catecholamines
- genetic testing
Investigations to consider
- 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)
without hypertensive crisis
Bridget Sinnott, MD
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.
Betul A. Hatipoglu, MD
Clinical Endocrinologist and Research Scientist
Department of Endocrinology, Diabetes, and Metabolism
BAH declares that she has no competing interests.
- Anxiety and panic attacks
- Essential or intractable hypertension
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma
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