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.


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.
Bourdeaut
Franck
F
Institut Curie, Département de Pédiatrie, Paris, France. franck.bourdeaut@curie.fr
de Carli
Emilie
E
Timsit
Sandra
S
Coze
Carole
C
Chastagner
Pascal
P
Sarnacki
Sabine
S
Delattre
Olivier
O
Peuchmaur
Michel
M
Rubie
Hervé
H
Michon
Jean
J
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*
D014660:Vasoactive Intestinal Peptide