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

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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.

Related collections

Most cited references 26

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Is Open Access

Data Resource Profile: The National Health Information Database of the National Health Insurance Service in South Korea

Sang Cheol Seong, Yeon-Yong Kim, Young-Ho Khang … (2016)

Data resource basics The National Health Information Database (NHID) is a public database on health care utilization, health screening, socio-demographic variables, and mortality for the whole population of South Korea, formed by the National Health Insurance Service. The population included in the data is over 50 million, and the participation rate in the health screening programs was 74.8% in 2014. The NHID covers data between 2002 and 2014. Those insured by NHI pay insurance contributions and receive medical services from their health care providers. The NHIS, as the single insurer, pays costs based on the billing records of health care providers (Figure 1). To govern and carry out these processes in the NHI, the NHIS built a data warehouse to collect the required information on insurance eligibility, insurance contributions, medical history, and medical institutions. In 2012, the NHIS formed the NHID using information from medical treatment and health screening records and eligibility data from an existing database system. Figure 1. The governance of the National Health Insurance of South Korea. Data collected The eligibility database includes information about income-based insurance contributions, demographic variables, and date of death. The national health screening database includes information on health behaviors and bio-clinical variables. The health care utilization database includes information on records on inpatient and outpatient usage (diagnosis, length of stay, treatment costs, services received) and prescription records (drug code, days prescribed, daily dosage). The long-term care insurance database includes information about activities of daily living and service grades. The health care provider database includes data about the types of institutions, human resources, and equipment. In the NHID, de-identified join keys replacing the personal identifiers are used to interlink these databases. Data resource use Papers published covered various diseases or health conditions like infectious diseases, cancer, cardiovascular diseases, hypertension, diabetes mellitus, and injuries and risk factors such as smoking, alcohol consumption, and obesity. The impacts of health care and public health policies on health care utilization have been also explored since the data include all the necessary information reflecting patterns of health care utilization. Reasons to be cautious First, information on diagnosis and disease may not be optimal for identifying disease occurrence and prevalence since the data have been collected for medical service claims and reimbursement. However, the NHID also collects prescription data with secondary diagnosis, so the accuracy of the disease information can be improved. Second, the data linkage with other secondary national data is not widely available due to privacy issues in Korea. Governmental discussions on the statutory reform of data linkage using the NHID are under way. Collaboration and data access Access to the NHID can be obtained through the Health Insurance Data Service home page (http://nhiss.nhis.or.kr). An ethics approval from the researchers’ institutional review board is required with submission of a study proposal, which is reviewed by the NHIS review committee before providing data. Further inquiries on data use can be obtained by contacting the corresponding author. Funding and competing interests This work was supported by the NHIS in South Korea. The authors declare no competing interests.

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Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators.

Montserrat Ayala-Ramirez, Lei Feng, Marcella M Johnson … (2011)

Pheochromocytomas and sympathetic paragangliomas are rare neuroendocrine tumors for which no precise histological or molecular markers have been identified to differentiate benign from malignant tumors. The aim was to determine whether primary tumor location and size are associated with malignancy and decreased survival. We performed a retrospective chart review of patients with either pheochromocytoma or sympathetic paraganglioma. The study group comprised 371 patients. Overall survival and disease-specific survival were analyzed according to tumor size and location. Sixty percent of patients with sympathetic paragangliomas and 25% of patients with pheochromocytomas had metastatic disease. Metastasis was more commonly associated with primary tumors located in the mediastinum (69%) and the infradiaphragmatic paraaortic area, including the organ of Zuckerkandl (66%). The primary tumor was larger in patients with metastases than in patients without metastatic disease (P < 0.0001). Patients with sympathetic paragangliomas had a shorter overall survival than patients with pheochromocytomas (P < 0.0001); increased tumor size was associated with shorter overall survival (P < 0.001). Patients with sympathetic paragangliomas were twice as likely to die of disease than patients with pheochromocytomas (hazard ratio = 1.93; 95% confidence interval = 1.20-3.12; P = 0.007). As per multivariate analysis, the location of the primary tumor was a stronger predictor of metastases than was the size of the primary tumor. The size and location of the primary tumor were significant clinical risk factors for metastasis and decreased overall survival duration. These findings delineate the follow-up and treatment for these tumors.

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Prospective study on the prevalence of secondary hypertension among hypertensive patients visiting a general outpatient clinic in Japan.

Jun-ichi Saito, Masao Omura, Y Kakuta … (2004)

Secondary hypertension (SH) including endocrine hypertension has been reported to be uncommon. We estimated the prevalence of SH among hypertensive patients. We prospectively studied 1,020 hypertensive patients. As an initial screening, we measured plasma aldosterone concentration, plasma renin activity, serum cortisol concentration and plasma catecholamine concentration and conducted abdominal ultrasonography (US). As a secondary screening, we performed furosemide plus upright test, captopril renography, dexamethasone suppression test, 24-h urine catecholamine measurement and abdominal CT. Finally, primary aldosteronism with the exception of idiopathic hyperaldosteronism, pheochromocytoma, and Cushing's syndrome were diagnosed by histopathological examination of surgical specimens. Idiopathic hyperaldosteronism was clinically diagnosed by adrenocorticotrophic hormone (ACTH)-stimulated adrenal venous sampling and renovascular hypertension by renal arteriography. There were 61 patients with primary aldosteronism, 5 with renovascular hypertension, 11 with Cushing's syndrome, 10 with preclinical Cushing's syndrome and 6 with pheochromocytoma, and the prevalence of SH was 9.1% among 1,020 hypertensive patients. In 76 (82%) of 93 patients with SH, hypertension was cured or improved after unilateral adrenalectomy, transsphenoidal pituitary adenectomy or percutaneous transluminal angioplasty. With the exception of US and CT, all initial and secondary screening tests were found to be sensitive and specific for differentiating SH from essential hypertension (EH). In conclusion, the measurement of various hormone concentrations was very sensitive for ruling out SH--a condition for which, in the present study, there were few specific signs or symptoms--while CT and US examinations were not always useful for differentiating SH from EH. The prevalence of curable SH among hypertensive subjects was higher in this study, which was conducted by our simple method of screening tests, than in previous reports. Hypertensive patients should be screened for SH and the underlying disease treated appropriately to avoid long-term use of antihypertensive drugs and risks of atherosclerotic complications.

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Author and article information

Journal

Journal ID (nlm-ta): Endocrinol Metab (Seoul)

Journal ID (iso-abbrev): Endocrinol Metab (Seoul)

Journal ID (publisher-id): ENM

Title: Endocrinology and Metabolism

Publisher: Korean Endocrine Society

ISSN (Print): 2093-596X

ISSN (Electronic): 2093-5978

Publication date (Print): March 2020

Publication date (Electronic): 19 March 2020

Volume: 35

Issue: 1

Pages: 157-164

Affiliations

[1 ]Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.

[2 ]Department of Biostatistics, Korea University College of Medicine, Seoul, Korea.

[3 ]Division of Endocrinology and Metabolism, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.

[4 ]Division of Endocrinology and Metabolism, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.

Author notes

Corresponding author: Sin Gon Kim. Division of Endocrinology and Metabolism, Department of Internal Medicine, Korea University College of Medicine, 73 Inchon-ro, Seongbuk-gu, Seoul 02841, Korea. Tel: +82-2-920-5890, Fax: +82-2-922-5974, k50367@ 123456korea.ac.kr

^*These authors contributed equally to this work.

Author information

Jung Hee Kim https://orcid.org/0000-0003-1932-0234

Hyemi Moon https://orcid.org/0000-0002-2586-1611

Sin Gon Kim https://orcid.org/0000-0002-7430-3675

Article

DOI: 10.3803/EnM.2020.35.1.157

PMC ID: 7090309

PubMed ID: 32207276

SO-VID: 8dce0d95-2ee4-41cc-bc80-99e9550ea863

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 24 October 2019

Date revision received : 28 December 2019

Date accepted : 28 January 2020

Funding

Funded by: Korea Health Industry Development Institute, CrossRef https://doi.org/10.13039/501100003710;