title: Desmoplastic infantile ganglioglioma: a potentially malignant tumor?
abstract: Desmoplastic infantile ganglioglioma is a rare intracranial tumor of early childhood with a usually excellent prognosis despite malignant features both radiologically and histologically. We present the case of a desmoplastic infantile ganglioglioma with histologically highly anaplastic features and both intracerebral and pial metastases. After partial resection the tumor was rapidly progressive and new metastases appeared. A combination of vincristine and carboplatinum was used according to the Low Grade Glioma Protocol of the International Society of Pediatric Oncology, with a temporary good response. When histologically characterized by highly anaplastic features, it seems the biologic behavior of this tumor remains uncertain. The aggressive behavior and the responsiveness to chemotherapy in this case may challenge the belief in the benign nature of these rare tumors.
authors.affiliation: Department of Pathology, University of Leuven, Belgium.
authors.forename: K
authors.initials: K
authors.lastname: De Munnynck
authors.forename: S
authors.initials: S
authors.lastname: Van Gool
authors.forename: F
authors.initials: F
authors.lastname: Van Calenbergh
authors.forename: Ph
authors.initials: P
authors.lastname: Demaerel
authors.forename: A
authors.initials: A
authors.lastname: Uyttebroeck
authors.forename: G
authors.initials: G
authors.lastname: Buyse
authors.forename: R
authors.initials: R
authors.lastname: Sciot
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D016190:Carboplatin / Q000008:administration & dosage; D002675:Child, Preschool; D003893:Desmin; D017809:Fatal Outcome; D005260:Female; D018303:Ganglioglioma / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D009363:Neoplasm Proteins / Q000032:analysis; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014408:Biomarkers, Tumor; D003893:Desmin; D009363:Neoplasm Proteins; D014750:Vincristine; D016190:Carboplatin