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Journal of Neurosurgical Sciences 2000 Settembre;44(3):150-4

lingua: Inglese

Desmoplastic non-infantile ganglioglioma. Case report

Marti A. 2, Almostarchid B. 2, Maher M. 1, Saidi A. 1

1 Department of Pathology and Neuropathology, Hôpital des Spécialités Oto-Neuro-Ophtalmologiques (ONO), Rabat, Morocco;
2 Department of Neurosurgery. Hôpital Avicenne, CHU Ibn Sina, Rabat


Desmoplastic gan­gli­o­gli­o­mas ­are ­rare ­mixed ­glial ­and neu­ro­nal cere­bral ­tumors, espe­cial­ly ­described in ­infants ­below 4 ­years of ­age ­but excep­tion­al cas­es ­have ­been report­ed in ­young ­adults. These ­tumors ­are gen­er­al­ly loc­al­ised in parie­tal or tem­po­ral ­lobes, ­present as a ­large cys­tic ­lesion ­with periph­er­al con­trast enhance­ment. They ­also ­have char­ac­ter­is­tic his­to­log­i­cal fea­tures: exten­sive desmo­pla­sia ­and tumo­ral ­cells of var­i­able ­size exhib­it­ing immu­no­his­to­chem­i­cal ­and ultra­struc­tu­ral fea­tures of ­glial ­and neu­ro­nal dif­fe­ren­ti­a­tion. Total sur­gi­cal remov­al is suf­fi­cient ­for ­the treat­ment of ­these ­tumors ­and no radio­ther­a­py or chem­o­ther­a­py ­are indi­cat­ed if com­plete resec­tion is ­achieved.
We ­report a ­case of des­mo­plas­tic gan­gli­o­gli­o­ma in a 19-­year-­old ­male. This ­tumor pre­sent­ed as a ­large parie­to- tem­po­ral cys­tic ­lesion ­with ­rimmed con­trast enhance­ment. At his­to­log­i­cal exam­ina­tion, ­this ­tumor exhib­it­ed exten­sive desmo­pla­sia ­and com­prised 2 ­types of tumo­ral ­cells: ­small ­cells ­with ­round ­nuclei, pos­i­tive ­for ­NSE, neu­ro­fil­a­ments ­and syn­ap­toph­y­sin ­and some­times pre­sent­ing typ­i­cal mor­pho­log­i­cal fea­tures of neu­ro­nal dif­fe­ren­ti­a­tion, ­and ­large ­cells ­with abun­dant eosin­o­phil­ic strong­ly stain­ing ­for ­GFAP. This obser­va­tion emphaz­is­es on ­the ­fact ­that des­mo­plas­tic gan­gli­o­gli­o­ma ­can no ­more be con­sid­ered as a spe­cif­ic ­entity of infan­cy ­and ­must be ­well rec­og­nised ­even in ­young ­adults ­because it ­may be mis­di­ag­nosed as malig­nant gli­o­ma.

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