Pheochromocytoma epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]Mohammed Abdelwahed M.D[3]

Overview

The incidence of pheochromocytoma ranges 0.2-0.8 per 100,000 persons. The median age at diagnosis is 24.9 years in familial cases and 43.9 years in sporadic cases. Both men and women are affected equally by pheochromocytoma.

Epidemiology and Demographics

Incidence

  • In the USA, the incidence of pheochromocytoma ranges from 0.2-0.8 per 100,000 persons.[1] [2]
  • Annually reported cases range from 500 to 1600 in the United States.[3]
  • Autopsy studies have discovered a higher number of cases than the actual prevalence rates. Ten percent of pheochromocytomas cases are discovered by chance. 

Prevalence

  • In the USA, the prevelance of pheochromocytoma ranges from 0.2-0.8 per 100,000 persons.[1]
  • The prevalence of pheochromocytoma in patients with hypertension in general outpatient clinics ia about 0.1%. [4]
  • The prevalence of pheochromocytoma is approximately 1.7% in children with hypertension.
  • About 5% of patients with incidentally discovered adrenal masses on imaging actually have pheochromocytoma.
  • The prevalence of pheochromocytoma in individuals carrying a germline mutation in pheochromocytoma susceptibility genes may be around 50%.

Case-fatality rate/Mortality rate

  • The incidence of pheochromocytoma ranges from 0.2-0.8 per 100,000 persons with a perioperative case-fatality rate/mortality rate of about 2.4%.[1] [5]
  • Metastatic PPGLs were associated with a 2.40-fold higher risk of mortality than non-metastatic PPGLs (95% confidence interval, 1.38 to 4.17; P=0.002). [6]

Age

  • Patients of all age groups may develop pheochromocytoma. Approximately 10% occur in children.
  • The median age at diagnosis is 40 years.
  • The average age at diagnosis is 24.9 years in hereditary cases and 43.9 years in sporadic cases.[1]
  • Hereditary tumors present at a younger age than sporadic.

Race

  • There is no racial predilection to pheochromocytoma. [7]

Gender

  • Pheochromocytoma affects men and women are equally. [1]

Region

  • Pheochromocytoma is a rare disease that tends to affect all populations equally.

Developed Countries

Developing Countries

References

  1. 1.0 1.1 1.2 1.3 1.4 National Cancer Institute. Physician Data Query Database 2015. National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
  2. Jameson, J (2017). Harrison's Principles of Internal Medicine 19th Edition and Harrison's Manual of Medicine 19th Edition VAL PAK. New York: McGraw-Hill Medical. ISBN 978-1260128857.
  3. Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K; et al. (2010). "The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer". Pancreas. 39 (6): 775–83. doi:10.1097/MPA.0b013e3181ebb4f0. PMC 3419007. PMID 20664475.
  4. Jameson, J (2017). Harrison's Principles of Internal Medicine 19th Edition and Harrison's Manual of Medicine 19th Edition VAL PAK. New York: McGraw-Hill Medical. ISBN 978-1260128857.
  5. Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G (2001). "Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center". J Clin Endocrinol Metab. 86 (4): 1480–6. doi:10.1210/jcem.86.4.7392. PMID 11297571.
  6. Kim JH, Moon H, Noh J, Lee J, Kim SG (2020). "Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service". Endocrinol Metab (Seoul). 35 (1): 157–164. doi:10.3803/EnM.2020.35.1.157. PMC 7090309 Check |pmc= value (help). PMID 32207276 Check |pmid= value (help).
  7. DE GRAEFF J, HORAK BJ (1964). "THE INCIDENCE OF PHAEOCHROMOCYTOMA IN THE NETHERLANDS". Acta Med Scand. 176: 583–93. doi:10.1111/j.0954-6820.1964.tb00661.x. PMID 14223534.


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