Differences in Clinical Outcomes Between Patients With Incidentally Detected and Clinically Detected Pheochromocytomas and Paragangliomas: A Retrospective Multicenter Study

Abstract

Objective With the increasing use of imaging, a substantial proportion of pheochromocytomas and paragangliomas (PPGLs) are detected incidentally. However, data comparing the clinical outcomes of patients with incidentally and clinically detected PPGLs remain limited. Therefore, we compared them in this study.Methods This multicenter retrospective study included 426 patients. Patients were classified as having incidentally or clinically detected PPGLs. Clinical, laboratory and genetic features; tumour stage; persistent/recurrent disease; disease-free survival (DFS); and overall survival were compared between patients with incidentally and clinically detected PPGLs.Results Among the patients, 216 had incidentally detected, and 210 had clinically detected PPGLs. Persistent/recurrent disease was diagnosed in 47 (11%) patients. Patients with incidentally detected PPGLs were older and more normotensive than those with clinically detected PPGLs. Hypertension, hereditary diseases, and persistent/recurrent diseases were more common in patients with clinically detected PPGLs. However, DFS did not differ between the two groups. Only hereditary disease and advanced tumour stage were associated with short DFS and were independent predictors of persistent/recurrent disease. Incidentally detected PPGLs were associated with low overall survival, a fact that could be confounded by the age.Conclusion The detection method was neither associated with short DFS nor a predictor of persistent/recurrent disease, whereas hereditary disease and tumour stage were associated with short DFS and were independent predictors of persistent/recurrent disease in patients with PPGLs. Therefore, genetic analysis, staging, and close follow-up are necessary to determine persistent/recurrent disease and develop individualised treatment and follow-up plans, irrespective of the detection method.