Overall survival, late mortality, and cancer-directed surgery among children and adolescents with ultra-rare pediatric pancreatoblastoma in the United States, 1975–2018

Background: Pediatric pancreatoblastoma is an extremely rare malignant tumor, posing diagnostic and treatment difficulties for pediatric surgeons. Using the Surveillance, Epidemiology, and End Results (SEER) database, we present an up-to-date report of the epidemiology, clinicopathological features, survival rates, and prognosis of pancreatoblastoma in pediatric patients. Methods: All pediatric patients diagnosed with pancreatoblastoma between 1975 and 2018 were identified in the SEER registries (SEER 8 registries and SEER 17 registries). We conducted a survival analysis to assess overall survival and 1- and 5-year late mortality rates. Descriptive statistics and log-rank test were performed. Results: A total of 22 children and adolescents with pancreatoblastoma were identified. In this cohort, 12 of 22 were male (54.55%), 14 were White (63.64%), and 11 were diagnosed between the ages of 1 and 4 years (50.0%). Among the 22 patients, 11 (50.0%) had distant metastases, whereas 7 (31.82%) had localized, and 4 (18.18%) had a regional disease. A total of 5 children and adolescents died during the study period, with cumulative survival rates of 14 of 17 (82.35%) and 10 of 11 (90.95%) among 1- and 5-year survivors, respectively. Cancer-directed surgery was significantly associated with an increased life expectancy (log-rank test, P = .018). Conclusion: Pediatric pancreatoblastoma is a rare entity. Cases that underwent surgery had a greater likelihood of overall survival and reduced late mortality.