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A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children
Konstantinos S Mylonas1, Dimitrios Nasioudis2, Diamantis I Tsilimigras3
1Department of Pediatric Surgery, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA; Surgery Working Group, Society of Junior Doctors, Menelaou 5 Street, 15123, Athens, Greece.
Insights
Malignant pancreatic tumors are rare in children. Cancer-directed surgery (CDS) significantly improves survival outcomes for pediatric patients with these rare cancers.
Area of Science:
- Pediatric Oncology
- Cancer Epidemiology
- Tumor Biology
Background:
- Pancreatic malignancies are exceptionally rare in pediatric populations.
- Understanding their clinicopathological characteristics is crucial for improving outcomes.
Purpose of the Study:
- To analyze the clinicopathological features of pediatric malignant pancreatic tumors.
- To determine the prognostic factors influencing survival in this cohort.
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results (SEER) database (1973-2013).
- Identified pediatric patients diagnosed with pancreatic malignancies.
- Employed Kaplan-Meier and Cox proportional hazards models for survival analysis.
Main Results:
- 114 pediatric patients with pancreatic tumors were identified.
- Neuroendocrine tumors were the most common subtype (35.1%); pancreatoblastoma was more frequent in younger children.
- Cancer-directed surgery (CDS) was the sole factor significantly associated with reduced mortality (HR: 5.1).
Conclusions:
- Pediatric pancreatic malignancies exhibit variable prognoses.
- Cancer-directed surgery is independently linked to superior survival in pediatric patients with pancreatic cancer.
Purpose:
To examine the clinicopathological characteristics and prognosis of pediatric patients with malignant pancreatic tumors in a population-based cohort.
Methods:
The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify all pediatric patients with malignant pancreatic tumors, diagnosed between 1973 and 2013. Kaplan-Meier analysis was performed to determine median and five-year overall survival (OS) rates. Univariate survival analysis was executed using the log-rank test. Cox proportional hazards model was used to identify variables independently associated with mortality.
Results:
A total of 114 patients with pancreatic malignancies were identified. Median patient age was 16years and the majority of patients were white (64%) females (61.4%). The most prevalent histologic subtype was neuroendocrine tumors (35.1%), whereas pancreatoblastoma was more common during the first decade of life (P<0.001). Distant metastases were noted in 41.7% of the patients, while 33.3% and 25% had localized and regional disease respectively. Five-year OS rates were 77%, 66.4% and 64.8% for patients with pancreatoblastoma, neuroendocrine and epithelial tumors respectively. No death was observed in the solid pseudopapillary tumor group. Only history of having cancer-directed surgery (CDS) was significantly associated with lower overall mortality (HR: 5.1, 95% CI: 2.1, 12.4).
Conclusion:
Pancreatic malignancies are rare in children. Their prognosis is variable and only CDS was independently associated with superior survival.
Evidence Rating/Classification:
Prognosis study, Level II.
