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

Abstract

Background: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors originating from chromaffin cells. PP-GLs 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 personyears. 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.

Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force

“…Patients with metastatic PPGLs have high morbidity and mortality rates due to the excessive catecholamines, and patients with metastatic PPGLs have a significantly lower 5-year survival rate compared to patients without metastases (90% vs. 60%) [ 31 , 32 ]. Similarly, a recent nationwide population-based epidemiological study in South Korea showed the hazard ratio for mortality of patients with metastatic PPGLs was twice higher compared to patients without metastatic PPGLs [ 1 ]. The common metastatic sites of metastatic PPGLs are the regional and distant lymph nodes, bone, liver, and lungs [ 33 , 34 ].…”

“…Pheochromocytomas and paragangliomas (PPGLs) are rare chromaffin cells-derived tumors that originated from the adrenal medulla and the extra-adrenal sympathetic or parasympathetic paraganglia, respectively. The overall annual incidence of PPGLs is estimated to be two to eight cases per million, and the recent report in Korea showed the annual incidence of 1.8 cases per million [1][2][3][4].…”

Determinants of anxiety and depression among pheochromocytoma patients

“…[ 3 ] Despite their low prevalence, phaeochromocytomas are associated with a high mortality rate and several complications. [ 4 ]…”

Pathology of Paraganglioma and Pheochromocytoma: Review of Articles and Updates in Pathology

“…There are various reports in the literature regarding the male to female ratio. Some studies reported no significant difference in prevalence between the sexes, while findings from another study demonstrated a slight female preponderance [6,8].…”

“…A more recent study from Korea showed that the paragangliomas and pheochromocytomas have the prevalence of 2.13 per 100,000 persons and an age-standardized incidence of 0.18 per 100,000 person-years [6].…”