O feocromocitoma é uma afecção grave; a incidência anual na população em geral é inferior a 1 por 100,000 pessoas-ano.[11]Ebbehoj A, Stochholm K, Jacobsen SF, et al. Incidence and clinical presentation of pheochromocytoma and sympathetic paraganglioma: a population-based study. J Clin Endocrinol Metab. 2021 Apr 23;106(5):e2251-61.
https://academic.oup.com/jcem/article/106/5/e2251/6104764
http://www.ncbi.nlm.nih.gov/pubmed/33479747?tool=bestpractice.com
[12]Berends AMA, Buitenwerf E, de Krijger RR, et al. Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: a nationwide study and systematic review. Eur J Intern Med. 2018 May;51:68-73.
http://www.ncbi.nlm.nih.gov/pubmed/29361475?tool=bestpractice.com
[13]Plouin PF, Gimenez-Roqueplo AP. Pheochromocytomas and secreting paragangliomas. Orphanet J Rare Dis. 2006 Dec 8;1:49.
https://www.doi.org/10.1186/1750-1172-1-49
http://www.ncbi.nlm.nih.gov/pubmed/17156452?tool=bestpractice.com
[14]Leung AA, Pasieka JL, Hyrcza MD, et al. Epidemiology of pheochromocytoma and paraganglioma: population-based cohort study. Eur J Endocrinol. 2021 Jan;184(1):19-28.
https://www.doi.org/10.1530/EJE-20-0628
http://www.ncbi.nlm.nih.gov/pubmed/33112261?tool=bestpractice.com
[15]Kim JH, Moon H, Noh J, et al. Epidemiology and prognosis of pheochromocytoma/paraganglioma in Korea: A nationwide study based on the National Health Insurance Service. Endocrinol Metab (Seoul). 2020 Mar;35(1):157-64.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7090309
http://www.ncbi.nlm.nih.gov/pubmed/32207276?tool=bestpractice.com
Uma incidência anual de 500-1600 casos de feocromocitoma ou paraganglioma (PPGL) foi relatada nos EUA.[16]Chen H, Sippel RS, O'Dorisio MS, et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010 Aug;39(6):775-83.
https://www.doi.org/10.1097/MPA.0b013e3181ebb4f0
http://www.ncbi.nlm.nih.gov/pubmed/20664475?tool=bestpractice.com
Estima-se que a prevalência de PPGL nos EUA esteja entre 1:2500 e 1:6500.[16]Chen H, Sippel RS, O'Dorisio MS, et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas. 2010 Aug;39(6):775-83.
https://www.doi.org/10.1097/MPA.0b013e3181ebb4f0
http://www.ncbi.nlm.nih.gov/pubmed/20664475?tool=bestpractice.com
Em um estudo populacional realizado no município de Olmsted, nos EUA, a prevalência de feocromocitoma era de 8 por 100,000 habitantes (em 1/1/2017).[17]Ebbehoj A, Li D, Kaur RJ, et al. Epidemiology of adrenal tumours in Olmsted County, Minnesota, USA: a population-based cohort study. Lancet Diabetes Endocrinol. 2020 Nov;8(11):894-902.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7601441
http://www.ncbi.nlm.nih.gov/pubmed/33065059?tool=bestpractice.com
O feocromocitoma é uma causa rara de hipertensão secundária, com prevalência de <1% em clínicas ambulatoriais para tratamento da hipertensão.[2]Lenders JW, Duh QY, Eisenhofer G, et al; Endocrine Society. Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99(6):1915-42.
https://academic.oup.com/jcem/article/99/6/1915/2537399
http://www.ncbi.nlm.nih.gov/pubmed/24893135?tool=bestpractice.com
[10]Davison AS, Jones DM, Ruthven S, et al. Clinical evaluation and treatment of phaeochromocytoma. Ann Clin Biochem. 2018 Jan;55(1):34-48.
https://www.doi.org/10.1177/0004563217739931
http://www.ncbi.nlm.nih.gov/pubmed/29027806?tool=bestpractice.com
Estudos de autópsia indicam que 0.05% a 0.1% dos pacientes têm feocromocitomas, o que significa que muitos casos podem passar não diagnosticados, ou os pacientes podem ser assintomáticos.[10]Davison AS, Jones DM, Ruthven S, et al. Clinical evaluation and treatment of phaeochromocytoma. Ann Clin Biochem. 2018 Jan;55(1):34-48.
https://www.doi.org/10.1177/0004563217739931
http://www.ncbi.nlm.nih.gov/pubmed/29027806?tool=bestpractice.com
Até 14% dos pacientes com massas adrenais descobertas acidentalmente em exames de imagem anatômicos têm feocromocitoma.[10]Davison AS, Jones DM, Ruthven S, et al. Clinical evaluation and treatment of phaeochromocytoma. Ann Clin Biochem. 2018 Jan;55(1):34-48.
https://www.doi.org/10.1177/0004563217739931
http://www.ncbi.nlm.nih.gov/pubmed/29027806?tool=bestpractice.com
A prevalência é equivalente em homens e mulheres e é relatada em pessoas de todas as raças. O pico de incidência se dá entre a terceira e a quinta décadas de vida, com uma idade média de 43 anos no diagnóstico.[3]Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014 Jan-Feb;38(1):7-41.
https://www.cpcancer.com/article/S0147-0272(14)00002-6/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/24636754?tool=bestpractice.com
No entanto, cerca de 20% são identificadas em crianças, geralmente associadas a condições genéticas subjacentes.[3]Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014 Jan-Feb;38(1):7-41.
https://www.cpcancer.com/article/S0147-0272(14)00002-6/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/24636754?tool=bestpractice.com
[18]Moon JK, Mattei P. Pheochromocytoma and paraganglioma. Semin Pediatr Surg. 2020 Jun;29(3):150926.
http://www.ncbi.nlm.nih.gov/pubmed/32571511?tool=bestpractice.com
Até um terço dos casos de feocromocitoma estão associados a síndromes hereditárias, como neoplasia endócrina múltipla 2A e 2B, neurofibromatose do tipo 1 e síndrome de Von Hippel-Lindau.[2]Lenders JW, Duh QY, Eisenhofer G, et al; Endocrine Society. Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99(6):1915-42.
https://academic.oup.com/jcem/article/99/6/1915/2537399
http://www.ncbi.nlm.nih.gov/pubmed/24893135?tool=bestpractice.com
[3]Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014 Jan-Feb;38(1):7-41.
https://www.cpcancer.com/article/S0147-0272(14)00002-6/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/24636754?tool=bestpractice.com
[19]Liu P, Li M, Guan X, et al. Clinical syndromes and genetic screening strategies of pheochromocytoma and paraganglioma. J Kidney Cancer VHL. 2018 Dec 27;5(4):14-22.
http://www.ncbi.nlm.nih.gov/pubmed/30613466?tool=bestpractice.com