The high sensitivity of metaiodobenzylguanidine (MIBG) scintigraphy fo
r sympathomedullary tumors such as neuroblastoma and pheochromocytoma
is well documented. The specificity of MIBG scintigraphy for these tum
ors is also high but has been incompletely characterized for other neu
ral crest tumors and non-neural crest tumors of childhood. Methods: Th
e medical records and MIBG scans of all children who had undergone MIB
G scintigraphy for known or suspected neuroblastoma or pheochromocytom
a were retrospectively reviewed at five major referral centers. Those
patients found to have pathologies other than neuroblastoma or pheochr
omocytoma form the basis of this study. Results: One hundred children
with a total of 110 lesions met the inclusion criteria. All had negati
ve MIBG scans except 1 of 2 children with infantile myofibromatosis, 1
of 2 with neuroendocrine carcinomas, 1 of 2 with pancreaticoblastomas
and 1 of 10 with primitive neuroectodermal tumors. Conclusion: MIBG s
cintigraphy is highly specific for neuroblastoma and pheochromocytoma.
Only 4% (4/100) of nonsympathomedullary tumors (non-pheochromocytoma
and non-neuroblastoma) in childhood showed MIBG uptake, of which only
2% (2/100) were of non-neural crest origin.