AUTHOR=Zheng Lei , Gu Yalong , Silang Jiangcun , Wang Jinlong , Luo Feng , Zhang Baopeng , Li Chuanhong , Wang Feng 

TITLE=Prognostic Nomograms for Predicting Overall Survival and Cancer‐Specific Survival of Patients With Malignant Pheochromocytoma and Paraganglioma

JOURNAL=Frontiers in Endocrinology

VOLUME=Volume 12 - 2021

YEAR=2021

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2021.684668

DOI=10.3389/fendo.2021.684668

ISSN=1664-2392

ABSTRACT=Abstract
Background: Malignant pheochromocytoma and paraganglioma (PPGL) are rare tumors with few prognostic tools. This study aimed to construct nomograms for predicting 3-year and 5-year survival for patients with malignant PPGL.
Methods: The patient data was retrieved from the Surveillance, Epidemiology, and End Results (SEER) database. A total of 764 patients diagnosed with malignant PPGL from 1975 to 2016 were included in this study. The patients were randomly divided into two cohorts; the training cohort (n = 536) and the validation cohort (n = 228). Univariate analysis, Lasso regression, and multivariate Cox analysis were used to identify independent prognostic factors, which were then utilized to construct survival nomograms. The nomograms were used to predict 3-year and 5-year overall survival (OS) and cancer-specific survival (CSS) for patients with malignant PPGL. The prediction accuracy of the nomogram was assessed using the concordance index (C-index), receiver operating characteristic (ROC) curves and calibration curves. Decision curve analysis (DCAs) was used to evaluate the performance of survival models. 
Results: Age, gender, tumor type, tumor stage, or surgery were independent prognostic factors for OS in patients with malignant PPGL, while age, tumor stage, or surgery were independent prognostic factors for CSS (P<.05). Based on these factors, we successfully constructed the OS and CSS nomograms. The C-indexes were 0.747 and 0.742 for the OS and CSS nomograms, respectively. In addition, both the calibration curves and ROC curves for the model exhibited reliable performance.
Conclusion: We successfully constructed nomograms for predicting the OS and CSS of patients with   malignant PPGL. The nomograms could inform personalized clinical management of the patients.