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Pheochromocytoma

Última revisão: 21 Aug 2025
Última atualização: 29 Apr 2025

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • 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"
Detalhes completos

Outros fatores diagnósticos

  • orthostatic hypotension
  • hypercalcemia
  • Cushing syndrome
  • diarrhea
  • fever
  • papilledema
  • abdominal masses
  • tremors
Detalhes completos

Fatores de risco

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

Investigações diagnósticas

Primeiras investigações a serem solicitadas

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

Tests to avoid

  • plasma catecholamines
Detalhes completos

Investigações a serem consideradas

  • 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
Detalhes completos

Algoritmo de tratamento

Inicial

hypertensive crisis

AGUDA

without hypertensive crisis

Colaboradores

Autores

Bridget Sinnott, MD
Bridget Sinnott

Professor of Medicine

Medical College of Georgia

Augusta

GA

Declarações

BS declares that she has no competing interests.

Agradecimentos

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

Declarações

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.

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.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

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.
  • Pheochromocytoma images
  • Diagnósticos diferenciais

    • Anxiety and panic attacks
    • Essential or intractable hypertension
    • Hyperthyroidism
    Mais Diagnósticos diferenciais
  • Diretrizes

    • NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
    • American Association of Endocrine Surgeons guidelines for adrenalectomy
    Mais Diretrizes
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