A Predictive Nomogram for Early Death in Pheochromocytoma and Paraganglioma

BackgroundPheochromocytoma (PHEO) and paraganglioma (PGL) are relatively rare neuroendocrine tumors. The factors affecting patients with early death remain poorly defined. We aimed to study the demographic and clinicopathologic pattern and to develop and validate a prediction model for PHEO/PGL patients with early death.MethodsData of 800 participants were collected from the Surveillance Epidemiology and End Results (SEER) database as a construction cohort, while data of 340 participants were selected as a validation cohort. Risk factors considered included the year of diagnosis, age at diagnosis, gender, marital status, race, insurance status, tumor type, primary location, laterality, the presence of distant metastasis. Univariate and multivariate logistic regressions were performed to determine the risk factors. R software was used to generate the nomogram. Calibration ability, discrimination ability, and decision curve analysis were analyzed in both construction and validation cohorts.ResultsPHEO and PGL patients accounted for 54.3% (N=434) and 45.7% (N=366), respectively. More than half of tumors (N=401, 50.1%) occurred in the adrenal gland, while 16.9% (N=135) were in aortic/carotid bodies. For the entire cohort, the median overall survival (OS) was 116.0 (95% CI: 101.5-130.5) months. The multivariate analysis revealed that older age (versus age younger than 31; age between 31 and 60: OR=2.03, 95% CI: 1.03-4.03, P=0.042; age older than 60: OR=5.46, 95% CI: 2.68-11.12, P