VIP hypersecretion as primary or secondary syndrome in neuroblastoma: A retrospective study by the Société Française des Cancers de l'Enfant (SFCE)

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. Procedures 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. Conclusion 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. Pediatr Blood Cancer 2009;52:585–590. © 2009 Wiley‐Liss, Inc.