O feocromocitoma é uma afecção grave; a incidência anual na população em geral é inferior a 1 por 100,000 pessoas-ano.[13]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
[14]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
[15]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
[16]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
[17]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.[18]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.[18]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).[19]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.[12]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.[12]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.[12]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
[20]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
Aproximadamente 35% a 40% dos pacientes com feocromocitoma ou paraganglioma (conhecidos em conjunto como PPGL) apresentam uma mutação das linhas germinativas em um dos genes conhecidos de suscetibilidade ao PPGL, como RET (associado à neoplasia endócrina múltipla do tipo 2) ou VHL (associado à síndrome de Von Hippel-Lindau).[1]Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65.
http://www.ncbi.nlm.nih.gov/pubmed/31390501?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
[4]Fishbein L, Merrill S, Fraker DL, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. 2013 May;20(5):1444-50.
https://pmc.ncbi.nlm.nih.gov/articles/PMC4291281
http://www.ncbi.nlm.nih.gov/pubmed/23512077?tool=bestpractice.com
[5]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