title: Malignant pancreatic tumors in children: a single-institution series.
abstract: BACKGROUND
  Pancreatic tumors in children represent a very rare entity. We reviewed the clinical and pathologic features of pediatric patients with pancreatic tumors at a single institution.
  
  
  METHODS
  We conducted a retrospective review of cases diagnosed at the Hospital for Sick Children between 1986 and 2010.
  
  
  RESULTS
  Twenty-seven patients were diagnosed during the study period: 18 with solid pseudopapillary neoplasm (SPN), 6 with pancreatic endocrine neoplasia, and 3 with other tumors. Of the 27 children, 3 had associated syndromes, in specific tuberous sclerosis, von Hippel-Lindau, and polycystic ovarian syndrome. The most common symptoms were pain and vomiting in SPN and hypoglycemia and seizures in insulinomas. Magnetic resonance imaging and computed tomographic scan were equally accurate in determining size and site of origin of the tumor, and both were better than ultrasound. All patients underwent surgery (distal pancreatectomy in 14 cases, Whipple procedure in 8, other procedures in 5), which represented the only treatment for all but 3 cases. Seventeen patients (94%) with SPN are alive (median follow-up, 32 months), 3 of whom had positive margins. Two patients died: 1 male with SPN with malignant transformation and 1 with fibrosarcoma.
  
  
  CONCLUSIONS
  We describe the largest single-institution study of pediatric pancreatic tumors. Females with SPN have an excellent outcome, even in presence of positive margins, suggesting that limited surgical resection may be appropriate for these patients.
authors.affiliation: Department of Hematology/Oncology, Queen Paola Children's Hospital, Antwerpen, Belgium.
authors.forename: Machiel
authors.initials: M
authors.lastname: van den Akker
authors.forename: Paola
authors.initials: P
authors.lastname: Angelini
authors.forename: Glenn
authors.initials: G
authors.lastname: Taylor
authors.forename: Rose
authors.initials: R
authors.lastname: Chami
authors.forename: J Ted
authors.initials: JT
authors.lastname: Gerstle
authors.forename: Abha
authors.initials: A
authors.lastname: Gupta
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D010179:Pancreas / Q000473:pathology; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome