title: Exocrine pancreatic tumors in childhood in Germany.
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Department of Pediatric Surgery, Children's University Hospital Tuebingen, Hoppe-Seyler-Strasse3, Tuebingen, Germany. verenaellerkamp@googlemail.com
authors.forename: Verena
authors.initials: V
authors.lastname: Ellerkamp
authors.forename: Steven W
authors.initials: SW
authors.lastname: Warmann
authors.forename: Peter
authors.initials: P
authors.lastname: Vorwerk
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.forename: Joerg
authors.initials: J
authors.lastname: Fuchs
mesh_terms: D000293:Adolescent; D018267:Carcinoma, Acinar Cell / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D021441:Carcinoma, Pancreatic Ductal / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D010190:Pancreatic Neoplasms / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D012189:Retrospective Studies; D015996:Survival Rate