Endocrinol Metab.  2020 Mar;35(1):157-164. 10.3803/EnM.2020.35.1.157.

Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service

Affiliations
  • 1Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
  • 2Department of Biostatistics, Korea University College of Medicine, Seoul, Korea.
  • 3Division of Endocrinology and Metabolism, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.
  • 4Division of Endocrinology and Metabolism, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea. k50367@korea.ac.kr

Abstract

BACKGROUND
Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors originating from chromaffin cells. PPGLs are associated with a high mortality rate and several complications. To date, no epidemiological studies have been conducted on PPGLs in Asia. This study aimed to investigate the epidemiology and prognosis of PPGLs in Korea using nationwide data.
METHODS
Using the National Health Insurance Service Database, subjects with a principal diagnosis of PPGLs on two or more occasions between 2003 and 2014 who satisfied the operational definition of PPGLs were included. Incidence, prevalence, complications, metastasis, and mortality were investigated.
RESULTS
In total, 1048 subjects with a mean age of 47.6±16.1 years were included. There was no sex preponderance. The overall prevalence of PPGLs was 2.13 per 100,000 persons, and the overall age-standardized incidence rate was 0.18 per 100,000 person-years. Malignant PPGLs accounted for 17.7% (185 of 1,048) of cases, and 94 subjects exhibited metastasis at the time of diagnosis. Among initially non-metastatic PPGLs, 9.5% (nine of 954) eventually metastasized after a mean duration of 78.1±41.4 months. The 5-year survival rates for non-metastatic and metastatic PPGLs at diagnosis were 97% and 84%, respectively. Multivariable Cox regression models adjusted for covariates showed that 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).
CONCLUSION
PPGLs are rare in Korea, and the prognosis of these endocrine tumors varies depending on whether they are benign or malignant. This epidemiological study paves the way for further research on PPGLs.

Keyword

Pheochromocytoma; Paraganglioma; Epidemiology; Prevalence; Incidence; Prognosis; Mortality

MeSH Terms

Asia
Chromaffin Cells
Diagnosis
Epidemiologic Studies
Epidemiology*
Humans
Incidence
Korea*
Mortality
National Health Programs*
Neoplasm Metastasis
Paraganglioma
Pheochromocytoma
Prevalence
Prognosis*
Survival Rate

Figure

  • Fig. 1 Flow chart of study subjects. PPGLs, pheochromocytomas and paragangliomas.

  • Fig. 2 (A) Age and (B) sex distributions of study subjects with pheochromocytomas and paragangliomas. The sex of three subjects was not identified.

  • Fig. 3 The number of study subjects and the incidence rate of pheochromocytomas and paragangliomas by year.

  • Fig. 4 Kaplan-Meier survival curve for metastasis in non-metastatic pheochromocytomas and paragangliomas at diagnosis.

  • Fig. 5 Kaplan-Meier survival curve according to metastasis at diagnosis. PPGL, pheochromocytomas and paraganglioma.


Cited by  3 articles

A Brief Overview of the Epidemiology of Pheochromocytoma and Paraganglioma in Korea
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Endocrinol Metab. 2020;35(1):95-96.    doi: 10.3803/EnM.2020.35.1.95.

Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force
Eu Jeong Ku, Kyoung Jin Kim, Jung Hee Kim, Mi Kyung Kim, Chang Ho Ahn, Kyung Ae Lee, Seung Hun Lee, You-Bin Lee, Kyeong Hye Park, Yun Mi Choi, Namki Hong, A Ram Hong, Sang-Wook Kang, Byung Kwan Park, Moon-Woo Seong, Myungshin Kim, Kyeong Cheon Jung, Chan Kwon Jung, Young Seok Cho, Jin Chul Paeng, Jae Hyeon Kim, Ohk-Hyun Ryu, Yumie Rhee, Chong Hwa Kim, Eun Jig Lee
Endocrinol Metab. 2021;36(2):322-338.    doi: 10.3803/EnM.2020.908.

Big Data Research in the Field of Endocrine Diseases Using the Korean National Health Information Database
Sun Wook Cho, Jung Hee Kim, Han Seok Choi, Hwa Young Ahn, Mee Kyoung Kim, Eun Jung Rhee
Endocrinol Metab. 2023;38(1):10-24.    doi: 10.3803/EnM.2023.102.


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