Exocrine pancreatic tumors in childhood in Germany

BACKGROUND: Pancreatic tumors (PT) in childhood are rare. Standard therapeutic approaches are lacking. Our aim was to analyze treatment modalities and outcome in children with PT. PROCEDURE: Between 1980 and 2007, 55 patients with exocrine PT < 16 years old were registered. Data were obtained from the German Pediatric Tumour Registry. Medical records were evaluated and patient data were pseudonymized. RESULTS: Patient records of 29 children were available (9 male, 20 female, median age 11.2 years, range 3.1-16). In 18 patients a solid-pseudopapillary tumor (SPT) was diagnosed, in 7 patients a pancreatic carcinoma (P-CA) (5 acinar cell carcinoma (ACC), 2 ductal adenocarcinoma (DCA)), and in 4 patients a pancreatoblastoma (PBL). In 69% of the patients the initial radiological findings led to an incorrect tentative diagnosis. Initial histopathological diagnoses were differing from the reference pathology in 50% of the SPT and 45% of the P-CA. In the group of SPT survival rate was 100%; all patients underwent surgical resection. There were two cases of tumor relapse and one late secondary malignancy of the pancreas (DCA). In P-CA patients, survival rate was 14%, in the PBL group the survival rate was 25%. Concepts of chemotherapy, radiotherapy, and surgical intervention in P-CA and PBL were varying widely. CONCLUSIONS: In all cases of pediatric PT reference pathology and reference radiology should be involved. Standardized treatment concepts as well as prospective data registrations need to be entrenched.