title: VIP hypersecretion as primary or secondary syndrome in neuroblastoma: A retrospective study by the Société Française des Cancers de l'Enfant (SFCE).
abstract: BACKGROUND
  Neuroblastic tumors (NTs) are occasionally associated with watery diarrhea, due to Vasoactive Intestinal Peptide (VIP) secretion. Most reports are single cases and suggest a great homogeny within this sub-group of NTs.
  
  
  METHODS
  We conducted a retrospective analysis of the French experience of NTs associated with watery diarrhea due to VIP-secretion. VIP secretion was confirmed by seric dosage and/or immunohistochemistry.
  
  
  RESULTS
  Twenty-two patients met the diagnostic criteria between 1988 and 2007. Most of patients suffered from weight loss and metabolic disorders. In 16 cases, digestive symptoms preceded the diagnosis of the tumor ("Primary VIP secreting NTs"); 15 were localized and all showed a differentiated histology. Interestingly, in another 6 patients with high-risk NT, diarrhea occurred at the time of chemotherapy or retinoic acid therapy ("Secondary VIP secreting NTs"). Differentiation in response to treatment was documented in 4 cases. In all cases, only surgical excision of the tumor was able to control the digestive symptoms. Twenty children are alive and 13 are disease-free.
  
  
  CONCLUSIONS
  VIP secreting NTs are usually associated with differentiation; they can also secondarily arise from a high-risk tumor upon treatment. Primary surgery constitutes first-line treatment.
authors.affiliation: Institut Curie, Département de Pédiatrie, Paris, France. franck.bourdeaut@curie.fr
authors.forename: Franck
authors.initials: F
authors.lastname: Bourdeaut
authors.forename: Emilie
authors.initials: E
authors.lastname: de Carli
authors.forename: Sandra
authors.initials: S
authors.lastname: Timsit
authors.forename: Carole
authors.initials: C
authors.lastname: Coze
authors.forename: Pascal
authors.initials: P
authors.lastname: Chastagner
authors.forename: Sabine
authors.initials: S
authors.lastname: Sarnacki
authors.forename: Olivier
authors.initials: O
authors.lastname: Delattre
authors.forename: Michel
authors.initials: M
authors.lastname: Peuchmaur
authors.forename: Hervé
authors.initials: H
authors.lastname: Rubie
authors.forename: Jean
authors.initials: J
authors.lastname: Michon
mesh_terms: D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005602:France; D006801:Humans; D007223:Infant; D008297:Male; D009447:Neuroblastoma / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology* / Q000556:secondary; D012189:Retrospective Studies; D012955:Societies, Medical; D015996:Survival Rate; D014660:Vasoactive Intestinal Peptide / Q000378:metabolism*
chemical_list: D014660:Vasoactive Intestinal Peptide