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Scott R. VandenBerg, Estelle E. May, Lucien J. Rubinstein, Mary M. Herman, E. Perentes, S. A. Vinores, V. Peter Collins and T. S. Park

(Cases 32 to 35). 9 TABLE 1 Clinical features of desmoplastic infantile gangliogliomas Case No. (Accession No.) Sex & Age at Presentation Location of Tumor Treatment Survival After First Op Survival & Recurrence * 1 (C87) M, 9 mos rt frontoparietal resection 14 yrs dead, NR 2 (C1528) M, 3 mos rt frontoparietal resection, radiation 10.5 yrs alive, NR 3 (C2021) M, 10 mos lt parietal resection, radiation 10.5 yrs alive, NR 4 (C4002) M, 18 mos lt frontoparietal

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. Schnitt January 1987 66 1 50 57 10.3171/jns.1987.66.1.0050 Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”) Scott R. VandenBerg Estelle E. May Lucien J. Rubinstein Mary M. Herman E. Perentes S. A. Vinores V. Peter Collins T. S. Park January 1987 66 1 58 71 10.3171/jns.1987.66.1.0058 Temporoparietal craniopagus Richard D. Bucholz Kong-Woo Yoon Raymond E. Shively January 1987 66 1 72 79 10

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James T. Rutka, Jackson Hall, Jane R. Giblin, Dolores V. Dougherty, Michael S. B. Edwards, Robert Stern and Mark L. Rosenblum

, Fryling C, De Larco JE: Transforming growth factors produced by certain human tumor cells: polypeptides that interact with epidermal growth factor receptors. Proc Natl Acad Sci USA 77: 5258–5262, 1980 29. VandenBerg SR , May EE , Rubinstein LJ , et al : Desmo-plastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). J Neurosurg 66 : 58 – 71 , 1987 VandenBerg SR, May EE, Rubinstein LJ, et al: Desmo-plastic supratentorial neuroepithelial

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Mary E. Keohane, Scott W. Hall, Scott R. VandenBerg and Steven L. Gonias

Acad Sci USA 76: 4350–4354, 1979 38. VandenBerg SR , May EE , Rubinstein LJ , et al : Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). A report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66 : 58 – 71 , 1987 VandenBerg SR, May EE, Rubinstein LJ, et al: Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile

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Akiko Furuta, Hitoshi Takahashi, Fusahiro Ikuta, Kiyoshi Onda, Norio Takeda and Ryuichi Tanaka

considered that there were too few ganglion cells to qualify within the ganglioglioma group. It has been proposed that gangliocytomas and gan-gliogliomas arise from the dysgenetic neuronal cell nests and are of hamartomatous origin. Some have accompanied anomalies of a congenital nature, such as glioneuronal ectopias in the leptomeninges or temporal cortex. 8 VandenBerg, et al. , 9 reported 11 cases of “desmoplastic infantile ganglioglioma,” which is a special form of embryonal brain tumor exhibiting divergent astrocytic and ganglionic differentiation. They described

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Margaret R. Wacker, Philip H. Cogen, Joan E. Etzell, Laleh Daneshvar, Richard L. Davis and Michael D. Prados

symptoms. One tumor reported by Johannsson, et al. , 13 was incidentally noted at autopsy. Over time, anaplastic change may occur within the glial component, which may then become predominant. Ganglioblastoma developing at the site of a ganglioglioma has been reported in two cases, one 5 years after resection and radiation therapy 14 and one 23 years after biopsy and radiation therapy. 20 A subgroup of gangliogliomas, the desmoplastic infantile gangliogliomas, consists of large, partially cystic supratentorial masses presenting before the age of 18 months. 23

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Douglas C. Miller, Frederick F. Lang and Fred J. Epstein

-associated proteins. Trends Neurosci 11 : 241 – 242 , 1988 Trimble WS, Scheller RH: Molecular biology of synaptic vesicle-associated proteins. Trends Neurosci 11: 241–242, 1988 29. VandenBerg SR , May EE , Rubinstein LJ , et al : Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (“desmoplastic infantile gangliogliomas”). Report of 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 66 : 58 – 71 , 1987 VandenBerg SR, May EE, Rubinstein

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Ho-keung Ng and Wai-sang Poon

. VandenBerg SR : Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 3 : 275 – 281 , 1993 VandenBerg SR: Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 3: 275–281, 1993 26. Wolbach SB : Congenital rhabdomyoma of the heart. Report of a case associated with multiple nests of neuroglia tissue in the meninges of the spinal cord. J Med Res 16 : 495 – 520 , 1907 Wolbach SB: Congenital rhabdomyoma of the heart. Report

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Minoru Tanaka, Soichiro Shibui, Kazuhiro Nomura, Yukihiro Nakanishi, Tadashi Hasegawa and Takanori Hirose

), intracerebral rhabdomyosarcomas, desmoplastic infantile ganglioglioma, intracerebral meningioma, and solitary fibrous tumor. No immunospecific marker has been isolated for NSTs. However, immunohistochemical analysis is useful to distinguish malignant NSTs from other lesions. In the present case, absence of glial fibrillary acidic protein or neurofilament triplet polypeptides allowed us to rule out gliomas and desmoplastic infantile ganglioglioma. In the same way, absence of EMA, desmin, CD-34 and HMB-45, in combination with the features seen on electron microscopy studies

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Bertrand Baussard, Federico Di Rocco, Matthew R. Garnett, Nathalie Boddaert, Arielle Lellouch-Tubiana, Jacques Grill, Stephanie Puget, Thomas Roujeau, Michel Zerah and Christian Sainte-Rose

, 1998 26 Tamburrini G , Colosimo C Jr , Giangaspero F , Riccardi R , Di Rocco C : Desmoplastic infantile ganglioglioma . Childs Nerv Syst 19 : 292 – 297 , 2003 27 Weiner HL , Freed D , Woo HH , Rezai AR , Kim R , Epstein FJ : Intra-axial tumors of the cervicomedullary junction: surgical results and long-term outcome . Pediatr Neurosurg 27 : 12 – 18 , 1997 28 Zentner J , Wolf HK , Ostertun B , Hufnagel A , Campos MG , Solymosi L , : Gangliogliomas: clinical, radiological, and histopathological findings