Pheochromocytoma
Summary
Definition
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"
Other diagnostic factors
- orthostatic hypotension
- hypercalcemia
- Cushing syndrome
- diarrhea
- fever
- papilledema
- abdominal masses
- tremors
Risk factors
- multiple endocrine neoplasia type 2 (MEN2)
- Von Hippel-Lindau (VHL) disease
- SDHB, SDHC, and SDHD gene mutations
- neurofibromatosis type 1 (NF1)
Diagnostic tests
1st tests to order
- plasma free metanephrines or 24-hour urine fractionated metanephrines and normetanephrines
- genetic testing
Tests to avoid
- plasma catecholamines
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
Treatment algorithm
hypertensive crisis
without hypertensive crisis
Contributors
Authors
Bridget Sinnott, MD
Professor of Medicine
Medical College of Georgia
Augusta
GA
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.
Revisores
Betul A. Hatipoglu, MD
Clinical Endocrinologist and Research Scientist
Department of Endocrinology, Diabetes, and Metabolism
Cleveland Clinic
Cleveland
OH
Declarações
BAH declares that she has no competing interests.
Créditos aos pareceristas
Os tópicos do BMJ Best Practice são constantemente atualizados, seguindo os desenvolvimentos das evidências e das diretrizes. Os pareceristas aqui listados revisaram o conteúdo pelo menos uma vez durante a história do tópico.
Declarações
As afiliações e declarações dos pareceristas referem--se ao momento da revisão.
Referências
Principais artigos
Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65. Resumo
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.Texto completo Resumo
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Texto completo
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.Texto completo Resumo
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.Texto completo Resumo
Artigos de referência
Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
Diagnósticos diferenciais
- Anxiety and panic attacks
- Essential or intractable hypertension
- Hyperthyroidism
Mais Diagnósticos diferenciaisDiretrizes
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- American Association of Endocrine Surgeons guidelines for adrenalectomy
Mais Diretrizes
Conectar-se ou assinar para acessar todo o BMJ Best Practice
O uso deste conteúdo está sujeito ao nosso aviso legal