Incidence, Management, and Survival of Pancreatic Malignancies in Children: A Population-Based SEER Study.
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BACKGROUND: Pancreatic neoplasms are uncommon in children. We assessed the incidence, treatment, and survival of children with pancreatic malignancies, and determined factors associated with mortality using a large database. METHODS: The Surveillance, Epidemiology, and End Results 22 database (excluding IL and MA) was queried to identify pediatric patients diagnosed with pancreatic malignancies between 2000 and 2020. Incidence, demographics, treatment modalities, and survival data were compared between tumor groups. RESULTS: Pancreatic malignancies were identified in 300 patients (69 % female). Patients were classified by histology into solid pseudopapillary carcinoma (SPC; n = 144, 48 %), neuroendocrine (NET; n = 77, 25.7 %), epithelial (EP; n = 27, 9 %), pancreatoblastoma (PB; n = 30, 10 %), and non-epithelial/unknown (NE/U; n = 22, 7.3 %). Overall median age at diagnosis was 14, whereas median age for pancreatoblastoma was 4 (p < 0.001). Majority of SPCs (86.8 %) were diagnosed in females (p < 0.001). At diagnosis, 95 (56.2 %), 40 (23.7 %), and 34 (20.1 %) patients had localized, regional, and metastatic disease, respectively. Overall, 225 (86.2 %) patients underwent operation. Five-year overall survival rates at 5-years for each tumor group were significantly different (p < 0.001) at 100 %, 84.2 %, 69 %, 67.2 %, and 59 % for SPC, NET, NE/U, PB, and EP types, respectively. Age-adjusted incidence of pancreatic malignancies was 0.0424 per 100,000. Annual percent change in incidence from 2000 to 2020 was 9.9 [CI: (6.8,13.1), p < 0.001). Regional and distant disease, not undergoing operation, and certain tumor types were associated with increased mortality. CONCLUSION: Pancreatic malignancy incidence increased over the last 20 years. Tumor type (pancreatoblastoma, neuroendocrine, and epithelial), advanced disease stage, and not undergoing surgery were independently associated with increased mortality. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: III.