Desmoplastic infantile ganglioglioma with a malignant course.
Desmoplastic infantile gangliogliomas (DIGs) are rare supratentorial tumors that arise in infancy. Despite the large size of these lesions, the prognosis is generally considered favorable after gross-total resection (GTR); however, in incidental cases tumor progression has been described. The authors report on a child harboring a DIG with a high-grade primitive cell population and glial differentiation. The progressive clinical course was determined by this cell component in spite of GTR and adjuvant chemotherapy. The significance of the presence of a high-grade primitive tumor component in the context of DIG is discussed.
Hoving
Eelco W
EW
Departments of Neurosurgery, University Medical Center Groningen, Groningen, The Netherlands. e.w.hoving@nchir.umcg.nl
Kros
Johan M
JM
Groninger
Ellis
E
den Dunnen
Wilfred F A
WF
D018303:Ganglioglioma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007223:Infant; D019394:Ki-67 Antigen / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D019635:Neurosurgical Procedures / Q000379:methods; D015173:Supratentorial Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D044767:Ubiquitin-Protein Ligases / Q000378:metabolism
D005904:Glial Fibrillary Acidic Protein; D019394:Ki-67 Antigen; C513496:MIB1 ligase, human; D044767:Ubiquitin-Protein Ligases