Functionally active ganglioneuroma with increased plasma and urinary catecholamines and positive iodine 131-meta-iodobenzylguanidine scintigraphy.
Ganglioneuromas are usually considered not to be functionally active. Studies of their catecholamine excretory pattern and of their imaging by means of the adrenergic tracing agent 131-I-MIBG have been therefore sparse. We report on a case of secretory ganglioneuroma, as demonstrated by the increased urinary excretion of the catecholamine metabolites HVA and VMA, increased plasma dopamine and epinephrine levels, and positive 131-I-MIBG scintigraphy. We must therefore be aware that a functionally active tumor is not necessarily a neuroblastoma, and that the diagnosis should be biopsy proven.
Clerico
A
A
Department of Pediatrics, University La Sapienza, Rome, Italy.
Jenkner
A
A
Castello
M A
MA
Ciofetta
G
G
Lucarelli
C
C
Codini
M
M
D019797:3-Iodobenzylguanidine; D002395:Catecholamines / Q000378:metabolism*; D002648:Child; D004298:Dopamine / Q000378:metabolism; D004837:Epinephrine / Q000378:metabolism; D005260:Female; D005729:Ganglioneuroma / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000473:pathology; D006719:Homovanillic Acid / Q000378:metabolism; D006801:Humans; D007457:Iodine Radioisotopes; D007462:Iodobenzenes; D011877:Radionuclide Imaging; D012186:Retroperitoneal Neoplasms / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000473:pathology; D014642:Vanilmandelic Acid / Q000378:metabolism
D002395:Catecholamines; D007457:Iodine Radioisotopes; D007462:Iodobenzenes; D019797:3-Iodobenzylguanidine; D014642:Vanilmandelic Acid; D004298:Dopamine; D006719:Homovanillic Acid; D004837:Epinephrine