Pheochromocytoma

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 1 Apr 2025

Last updated: 29 Apr 2025

Pheochromocytoma is a rare neuroendocrine tumor of the adrenal glands. It can present 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 metanephrines and normetanephrines.

Treatment includes medical therapy for hypertension 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.

Definition

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.[1] Symptoms are usually episodic and tend to progress as the tumor grows.

The majority of these tumors are benign. About 80% to 85% arise in the adrenal gland, and the remainder are tumors derived from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia known as paragangliomas.[2] These tumors mostly develop sporadically. Approximately 35% to 40% of patients with pheochromocytoma or paraganglioma (together known as PPGL) have a germline mutation in one of the known PPGL susceptibility genes such as RET (associated with multiple endocrine neoplasia type 2) or VHL (associated with Von Hippel-Lindau syndrome).[1][3][4][5]

History and exam

Key diagnostic factors

headache
palpitations
diaphoresis
hypertension
hypertensive retinopathy
pallor
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"

Full details

Other diagnostic factors

orthostatic hypotension
hypercalcemia
Cushing syndrome
diarrhea
fever
papilledema
abdominal masses
tremors

Full details

Risk factors

multiple endocrine neoplasia type 2 (MEN2)
Von Hippel-Lindau (VHL) disease
SDHB, SDHC, and SDHD gene mutations
neurofibromatosis type 1 (NF1)

Full details

Diagnostic tests

1st tests to order

plasma free metanephrines or 24-hour urine fractionated metanephrines and normetanephrines
genetic testing

Full details

Tests to avoid

plasma catecholamines

Full details

Tests to consider

CBC
serum calcium
serum potassium
chromogranin A
clonidine suppression test
CT scan of the abdomen and pelvis
MRI of the abdomen and pelvis
18F-fluorodeoxyglucose (18F-FDG) PET/CT or somatostatin receptor-targeted (SSTR) PET/CT with 68Ga-DOTATATE tracer
I-123 metaiodobenzylguanidine (MIBG) scintigraphy

Full details

Treatment algorithm

INITIAL

hypertensive crisis

ACUTE

without hypertensive crisis

Contributors

Authors

Bridget Sinnott, MD

Professor of Medicine

Medical College of Georgia

Augusta

Disclosures

BS declares that she has no competing interests.

Acknowledgements

Dr Bridget Sinnott would like to gratefully acknowledge Dr Sidhbh Gallagher, a previous contributor to this topic.

Disclosures

SG declares that she has no competing interests.

Peer reviewers

Betul A. Hatipoglu, MD

Clinical Endocrinologist and Research Scientist

Department of Endocrinology, Diabetes, and Metabolism

Cleveland Clinic

Cleveland

Disclosures

BAH declares that she has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65. Abstract

Fishbein L, Del Rivero J, Else T, et al. The North American Neuroendocrine Tumor Society consensus guidelines for surveillance and management of metastatic and/or unresectable pheochromocytoma and paraganglioma. Pancreas. 2021 Apr 1;50(4):469-93.Full text Abstract

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Full text Abstract

Plouin PF, Amar L, Dekkers OM, et al; Guideline Working Group. European Society of Endocrinology clinical practice guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol. 2016 May;174(5):G1-10.Full text Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials
- Anxiety and panic attacks
- Essential or intractable hypertension
- Hyperthyroidism
More Differentials
Guidelines
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- American Association of Endocrine Surgeons guidelines for adrenalectomy
More Guidelines
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Pheochromocytoma

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to avoid

Tests to consider

Treatment algorithm

hypertensive crisis

without hypertensive crisis

Contributors

Authors

Bridget Sinnott, MD

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Betul A. Hatipoglu, MD

Disclosures

References

Key articles

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

Log in to access all of BMJ Best Practice