Desmoplastic infantile astrocytoma/desmoplastic infantile ganglioglioma

2006 ◽  
pp. 321-331 ◽  
Keyword(s):  
Desmoplastic Infantile Ganglioglioma ◽  
Desmoplastic Infantile Astrocytoma

scholarly journals LGG-36. DESMOPLASTIC INFANTILE GANGLIOGLIOMA (DIG) WITH A PPP1CB-ALK FUSION IN A 6-YEAR-OLD GIRL

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii373-iii373
Author(s):  
William McDonald ◽  
Mahmoud Nagib ◽  
Robert Jenkins ◽  
Cristiane Ida ◽  
Kevin Halling ◽  
...  
Keyword(s):  
Gene Fusion ◽  
Mapk Pathway ◽  
High Grade Glioma ◽  
Pediatric Brain Tumors ◽  
Molecular Entity ◽  
Low Grade ◽  
High Grade ◽  
Desmoplastic Infantile Ganglioglioma ◽  
Desmoplastic Infantile Astrocytoma ◽  
Exon 20

Abstract Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma (DIG) are benign glioneuronal tumors that typically occur in infants, involve the superficial cerebral cortex, and have an excellent prognosis. DIA/DIG are a distinct molecular entity based on DNA methylation profiling. BRAF600 mutations are frequently reported in DIG/DIA. A recent comprehensive genetic analysis of infantile hemispheric gliomas identified 2 unique groups: group 1 harbored alterations in the receptor tyrosine kinase (RTK) genes ALK, ROS1, NTRK, and MET and group 2 harbored alterations in the RAS/MAPK pathway. We report a case of a 6.5-year-old girl who presented with seizures and right homonymous hemianopia. MRI of her brain demonstrated a large cystic/solid left hemispheric mass with remodelling of the overlying skull, consistent with a long-standing process. She underwent a gross total resection (GTR) and pathology demonstrated a DIG with a PPP1CB-ALK gene fusion (exon 5 to exon 20) identified by RNA sequencing. She remains disease free 12 months following GTR. A literature review identified 4 reported cases of pediatric brain tumors with PPP1CB-ALK gene fusions including: a 3-month-old with a hemispheric high-grade glioma which recurred 4 years later and pathology showed mature ganglioglioma, with both tumors showing the identical PPP1CB-ALK gene fusion; a 10-month-old infant with a hemispheric low-grade glioma; an infant with a “congenital” hemispheric high-grade glioma; and a child with an astrocytoma with no further clinical data. PPP1CB-ALK gene fusion appears to be a rare oncogenic driver in gliomas of infancy, including DIG.

Desmoplastic Infantile Ganglioglioma

2002 ◽  
Vol 26 (11) ◽  
pp. 1515-1522 ◽  
Author(s):  
K. De Munnynck ◽  
S. Van Gool ◽  
F. Van Calenbergh ◽  
Ph. Demaerel ◽  
A. Uyttebroeck ◽  
...  
Keyword(s):  
Desmoplastic Infantile Ganglioglioma

Cytologic Features of Desmoplastic Infantile Ganglioglioma

2001 ◽  
Vol 45 (6) ◽  
pp. 1037-1042 ◽  
Author(s):  
Yukari Hasegawa ◽  
Yoko Hayabuchi ◽  
Ikuko Namba ◽  
Takako Watanabe ◽  
Keisuke Kato ◽  
...  
Keyword(s):  
Desmoplastic Infantile Ganglioglioma

Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features

2006 ◽  
Vol 48 (5) ◽  
pp. 617-621 ◽  
Author(s):  
M Cerda-Nicolas ◽  
C Lopez-Gines ◽  
R Gil-Benso ◽  
J Donat ◽  
R Fernandez-Delgado ◽  
...  
Keyword(s):  
Desmoplastic Infantile Ganglioglioma ◽  
Genetic Features

Desmoplastic (Infantile) Astrocytoma/Ganglioglioma (DIA/DIG)

2019 ◽  
pp. 1545-1552
Author(s):  
Serge Weis ◽  
Michael Sonnberger ◽  
Andreas Dunzinger ◽  
Eva Voglmayr ◽  
Martin Aichholzer ◽  
...  
Keyword(s):  
Desmoplastic Infantile Astrocytoma

scholarly journals Desmoplastic infantile ganglioglioma

2019 ◽  
Vol 32 ◽  
Author(s):  
Salma Kamoun ◽  
Heifa Azouz ◽  
Marwa Zemmali ◽  
Slim Haouet ◽  
Nidhameddine Kchir
Keyword(s):  
Desmoplastic Infantile Ganglioglioma

scholarly journals Diffusion, Perfusion, and Histopathologic Characteristics of Desmoplastic Infantile Ganglioglioma

2016 ◽  
Vol 10 (7) ◽  
Author(s):  
Chang Yueh Ho ◽  
Melissa Gener ◽  
Jose Bonnin ◽  
Stephen Francis Kralik
Keyword(s):  
Desmoplastic Infantile Ganglioglioma

Telomere associations in desmoplastic infantile ganglioglioma

1996 ◽  
Vol 92 (1) ◽  
pp. 4-7 ◽  
Author(s):  
Jonathan P. Park ◽  
Josiane R. Dossu ◽  
C.Harker Rhodes
Keyword(s):  
Desmoplastic Infantile Ganglioglioma

scholarly journals Desmoplastic Infantile Ganglioglioma —Case Report—

1993 ◽  
Vol 33 (7) ◽  
pp. 463-466 ◽  
Author(s):  
Toshimitsu AIDA ◽  
Hiroshi ABE ◽  
Terufumi ITOH ◽  
Kazuo NAGASHIMA ◽  
Kazuaki INOUE
Keyword(s):  
Case Report ◽  
Desmoplastic Infantile Ganglioglioma
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