Summary
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- cefaleia
- palpitações
- diaforese
- hipertensão
- retinopatia hipertensiva
- palidez
- intolerância à glicose/diabetes mellitus
- história familiar de distúrbios endócrinos
- história prévia de feocromocitoma
- taquiarritmia e infarto do miocárdio
- ataques de pânico ou "sensação de morte iminente"
Other diagnostic factors
- hipotensão ortostática
- hipercalcemia
- Síndrome de Cushing
- diarreia
- febre
- papiledema
- massas abdominais
- tremores
Risk factors
- Neoplasia endócrina múltipla tipo 2 (NEM2)
- doença de Von Hippel-Lindau (VHL)
- Mutações genéticas em SDHB, SDHC e SDHD
- neurofibromatose do tipo 1 (NF1)
Diagnostic investigations
1st investigations to order
- metanefrinas séricas livres ou metanefrinas e normetanefrinas urinárias de 24 horas fracionadas
- teste genético
Investigations to consider
- Hemograma completo
- cálcio sérico
- potássio sérico
- cromogranina A
- exame de supressão de clonidina
- tomografia computadorizada (TC) do abdome e da pelve
- ressonância nuclear magnética (RNM) do abdome e pelve
- PET-CT com 18F-fluordesoxiglucose (18F-FDG) ou PET-CT direcionada ao receptor de somatostatina (SSTR) com traçador 68Ga-DOTATATE
- cintilografia com I-123 metaiodobenzilguanidina (MIBG)
Treatment algorithm
crise hipertensiva
sem crise hipertensiva
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.
Peer reviewers
Betul A. Hatipoglu, MD
Clinical Endocrinologist and Research Scientist
Department of Endocrinology, Diabetes, and Metabolism
Cleveland Clinic
Cleveland
OH
Disclosures
BAH declares that she has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
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
- Ansiedade e ataques de pânico
- Hipertensão essencial ou intratável
- Hipertireoidismo
More DifferentialsGuidelines
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- American Association of Endocrine Surgeons guidelines for adrenalectomy
More Guidelines
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