Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients.

OBJECTIVE

The purpose of the study was to examine current incidence trends and outcomes for children with pancreatic malignancies.

METHODS

The Surveillance, Epidemiology, and End Results registry (1973-2004) was examined for pediatric patients with pancreatic malignancies (up to 19 years of age).

RESULTS

Malignant pancreatic neoplasms were identified in 58 patients. Females outnumbered males 1.9 to 1 (38 vs 20) for an age population-adjusted incidence of 0.021 and 0.015 per 100,000. Overall, 70% (n = 41) of patients were white. Asians had the highest incidence. Tumors were classified as exocrine (n = 31, 53.4%), endocrine (n = 19, 32.8%), or sarcomas (n = 5, 8.6%). Exocrine tumors included pancreatoblastoma (n = 10), solid-cystic tumor (SCT) (n = 10), ductal adenocarcinoma (DA) (n = 7), and acinar cell carcinoma (ACC) (n = 4). All SCTs and 80% of pancreatoblastomas were seen in females, whereas males had a higher incidence of DA 71% (P = .036). Ductal adenocarcinoma, SCT, acinar cell carcinoma, sarcomas, and endocrine tumors were more common in children older than 10 years, whereas pancreatoblastoma was more common in younger children (P = .045). Almost half of patients (n = 25) presented with distant metastasis; of these, 44% were endocrine tumors. Survival was significantly greater for female patients (P = .004) and for those who had surgery (P = .001) by both univariate and multivariate analysis. There was a significant difference in tumor type 15-year survival with DA having the worst (23%) and SCT the best (100%).

CONCLUSIONS

Pediatric pancreatic neoplasms are uncommon and carry a variable prognosis. Both female sex and surgery were independent predictors of improved survival.