Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service
BackgroundPheochromocytomas 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...
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| Format: | Article |
| Language: | English |
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Korean Endocrine Society
2020-03-01
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| Series: | Endocrinology and Metabolism |
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| Online Access: | https://e-enm.org/Synapse/Data/PDFData/2008ENM/enm-35-157.pdf |
| _version_ | 1818353186434449408 |
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| author | Jung Hee Kim Hyemi Moon Junghyun Noh Juneyoung Lee Sin Gon Kim |
| author_facet | Jung Hee Kim Hyemi Moon Junghyun Noh Juneyoung Lee Sin Gon Kim |
| author_sort | Jung Hee Kim |
| collection | DOAJ |
| description | BackgroundPheochromocytomas 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.MethodsUsing 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.ResultsIn 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).ConclusionPPGLs 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. |
| first_indexed | 2024-12-13T19:05:32Z |
| format | Article |
| id | doaj.art-bea5ac2559ef4995b63385088f4ff702 |
| institution | Directory Open Access Journal |
| issn | 2093-596X 2093-5978 |
| language | English |
| last_indexed | 2024-12-13T19:05:32Z |
| publishDate | 2020-03-01 |
| publisher | Korean Endocrine Society |
| record_format | Article |
| series | Endocrinology and Metabolism |
| spelling | doaj.art-bea5ac2559ef4995b63385088f4ff7022022-12-21T23:34:33ZengKorean Endocrine SocietyEndocrinology and Metabolism2093-596X2093-59782020-03-0135115716410.3803/EnM.2020.35.1.157Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance ServiceJung Hee Kim0Hyemi Moon1Junghyun Noh2Juneyoung Lee3Sin Gon Kim4Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, .KoreaDepartment of Biostatistics, Korea University College of Medicine, Seoul, .KoreaDivision of Endocrinology and Metabolism, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, .KoreaDepartment of Biostatistics, Korea University College of Medicine, Seoul, .KoreaDivision of Endocrinology and Metabolism, Department of Internal Medicine, Korea University College of Medicine, Seoul, .KoreaBackgroundPheochromocytomas 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.MethodsUsing 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.ResultsIn 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).ConclusionPPGLs 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.https://e-enm.org/Synapse/Data/PDFData/2008ENM/enm-35-157.pdfpheochromocytomaparagangliomaepidemiologyprevalenceincidenceprognosismortality |
| spellingShingle | Jung Hee Kim Hyemi Moon Junghyun Noh Juneyoung Lee Sin Gon Kim Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service Endocrinology and Metabolism pheochromocytoma paraganglioma epidemiology prevalence incidence prognosis mortality |
| title | Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service |
| title_full | Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service |
| title_fullStr | Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service |
| title_full_unstemmed | Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service |
| title_short | Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service |
| title_sort | epidemiology and prognosis of pheochromocytoma paraganglioma in korea a nationwide study based on the national health insurance service |
| topic | pheochromocytoma paraganglioma epidemiology prevalence incidence prognosis mortality |
| url | https://e-enm.org/Synapse/Data/PDFData/2008ENM/enm-35-157.pdf |
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