Prognostic factors of pancreatic tumors in children and adolescents: a population study based on the surveillance, epidemiology, and end results database

Abstract

Purpose: Pancreatic tumors in children are uncommon, and data is scarce. The purpose of this study is to examine the prognostic factors of pediatric pancreatic tumors in a population-based cohort.

Methods: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all pediatric patients with pancreatic tumors diagnosed between 1975 and 2018. The overall survival (OS) rates were determined using a Kaplan-Meier analysis. The log-rank test was used for univariate survival analysis. Cox proportional-hazards regression was used to determine the variables related to OS.

Results: We identified 195 children with pancreatic tumors, with a median age at diagnosis of 16 years. Tumors were classified as neuroendocrine tumors (33.8%), solid pseudopapillary tumors (SPTs) (32.3%), pancreatoblastoma (11.3%), and others (22.6%). Of the patients, 30.3% had distant metastases, and 69.7% had surgery. Pancreatoblastomas were more common in younger children, whereas solid pseudopapillary tumors were more common in female patients. Overall 1-year, 3-year, and 5-year survival rates for all patients were 90.3%, 79.2%, and 77.7%, respectively. The Cox proportional hazard regression revealed that SEER stage and surgery were significant independent predictors of overall survival.

Conclusions: Pancreatic tumors are rare in children, and overall survival is grim except for SPTs. SEER stage and surgery were determined to be the most relevant determinants of OS in our study.

Keywords: Neuroendocrine tumors; Pancreatic tumors; Solid pseudopapillary tumors; Survival.

Fig. 1

Kaplan–Meier analysis of OS in pediatric pancreatic tumors stratified by the SEER stage, grade, surgery, lymph nodes (LN) removed and chemotherapy. (A) Distant vs. Localized and regional, P < 0.001. (B) Low and moderately differentiated vs. Poorly and undifferentiated, P = 0.013. (C) Surgery vs. No surgery, P < 0.001. (D) LN removed vs. LN not removed, P = 0.001. (E) Chemotherapy vs. No chemotherapy, P < 0.001

Fig. 2

Kaplan–Meier analysis of OS in pediatric pancreatic tumors except SPT, when stratified by chemotherapy and surgery. Surgery-based regimens were associated with significantly improved the 5-y OS rate compared with other treatment regimens (P < 0.001)