title: From glioblastoma to gangliocytoma: an unforeseen but welcome shift in biological behavior.
abstract: The prognosis in children harboring a glioblastoma multiforme (GBM) is usually poor. Few GBMs in children, however, seem to respond quite well to adjuvant chemotherapy. The biological basis for such chemotherapy sensitivity remains uncertain. In this paper the authors report the case of a 2-month-old girl with a histologically confirmed GBM (WHO Grade IV) in whom chemotherapy was accompanied by differentiation of the malignant primary tumor into a typical gangliocytoma (WHO Grade I) showing ganglioid differentiation and expression of neuronal markers synaptophysin, neurofilament, and NeuN as well as a low Ki 67/MIB-1 proliferation index. Array-comparative genomic hybridization did not reveal genetic alterations in either specimen. Even though the underlying biological mechanisms remain to be elucidated, closer examination of frequency and prognostic significance of neuronal differentiation in pediatric GBMs within ongoing and future clinical trials may be warranted.
authors.affiliation: Institute of Neuropathology, University Hospital Münster, Heidelberg, Germany. jeibmann@uni-muenster.de
authors.forename: Astrid
authors.initials: A
authors.lastname: Jeibmann
authors.forename: Martin
authors.initials: M
authors.lastname: Hasselblatt
authors.forename: Stefan
authors.initials: S
authors.lastname: Pfister
authors.forename: Ronald
authors.initials: R
authors.lastname: Sträter
authors.forename: Angela
authors.initials: A
authors.lastname: Brentrup
authors.forename: Markus
authors.initials: M
authors.lastname: Holling
authors.forename: Thomas
authors.initials: T
authors.lastname: Niederstadt
authors.forename: Werner
authors.initials: W
authors.lastname: Paulus
authors.forename: Michael C
authors.initials: MC
authors.lastname: Frühwald
mesh_terms: D034961:Antigens, Nuclear / Q000378:metabolism; D000970:Antineoplastic Agents / Q000008:administration & dosage; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor; D001932:Brain Neoplasms / Q000473:pathology* / Q000628:therapy*; D016190:Carboplatin / Q000008:administration & dosage; D002454:Cell Differentiation; D049109:Cell Proliferation; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005729:Ganglioneuroma / Q000473:pathology* / Q000628:therapy*; D005909:Glioblastoma / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D006849:Hydrocephalus / Q000473:pathology; D007223:Infant; D009419:Nerve Tissue Proteins / Q000378:metabolism; D016900:Neurofilament Proteins / Q000378:metabolism; D019635:Neurosurgical Procedures; D020133:Reverse Transcriptase Polymerase Chain Reaction; D016708:Synaptophysin / Q000378:metabolism
chemical_list: D034961:Antigens, Nuclear; D000970:Antineoplastic Agents; D000972:Antineoplastic Agents, Phytogenic; D014408:Biomarkers, Tumor; D009419:Nerve Tissue Proteins; D016900:Neurofilament Proteins; D016708:Synaptophysin; C506808:neuronal nuclear antigen NeuN, human; D005047:Etoposide; D016190:Carboplatin