title: Pancreatic surgery for tumors in children and adolescents.
abstract: OBJECTIVE
  Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies.
  
  
  METHODS
  We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015.
  
  
  RESULTS
  Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5-18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7-10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma.
  
  
  CONCLUSIONS
  Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.
authors.affiliation: Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: Maria G
authors.initials: MG
authors.lastname: Sacco Casamassima
authors.affiliation: Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: Colin D
authors.initials: CD
authors.lastname: Gause
authors.affiliation: Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: Seth D
authors.initials: SD
authors.lastname: Goldstein
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
authors.forename: Fizan
authors.initials: F
authors.lastname: Abdullah
authors.affiliation: Division of Pediatric Radiology, The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Avner
authors.initials: A
authors.lastname: Meoded
authors.affiliation: Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Lukish
authors.affiliation: Department of Surgery, The Sol Goldman Pancreatic Research Center, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Wolfgang
authors.affiliation: Department of Surgery, The Sol Goldman Pancreatic Research Center, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: John
authors.initials: J
authors.lastname: Cameron
authors.affiliation: Division of Pediatric Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: David J
authors.initials: DJ
authors.lastname: Hackam
authors.affiliation: Department of Pathology, The Sol Goldman Pancreatic Research Center, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Ralph H
authors.initials: RH
authors.lastname: Hruban
authors.affiliation: Department of Surgery, Johns Hopkins All Children's Hospital, 601 5th Street South, Suite 501, Saint Petersburg, FL, 33701, USA. pc@jhmi.edu.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Colombani
mesh_terms: D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D018293:Cystadenoma, Serous / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D010180:Pancreatectomy / Q000379:methods*; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies
keywords: Children; Pancreatic neoplasm; Pancreatic neuroendocrine tumor; Pancreatoblastoma; Pediatric; Solid pseudopapillary tumor