mr- morphology and exact anatomic localisation of optic pathway gliomas in children with and without...
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![Page 1: MR- Morphology and exact Anatomic Localisation of Optic Pathway Gliomas in children with and without NF1 S. Leykamm¹², B. Bison², B. Thieme *, A.K. Gnekow](https://reader035.vdocuments.us/reader035/viewer/2022070305/5513cf645503466f748b4c0c/html5/thumbnails/1.jpg)
MR- Morphology and exact MR- Morphology and exact Anatomic Localisation of Optic Anatomic Localisation of Optic Pathway Gliomas in children Pathway Gliomas in children
with and without NF1with and without NF1
MR- Morphology and exact MR- Morphology and exact Anatomic Localisation of Optic Anatomic Localisation of Optic Pathway Gliomas in children Pathway Gliomas in children
with and without NF1with and without NF1
S. Leykamm¹², B. Bison², B. Thieme*, A.K. Gnekow*, L. Solymosi², M. Warmuth-Metz²
¹Klinik für Radiologische Diagnostik, RWTH Aachen,²Abt. für Neuroradiologie, Universität Würzburg,
*SIOP/HIT-LGG Studienzentrale Augsburg
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SIOP/HIT LGG-StudySIOP/HIT LGG-StudySIOP/HIT LGG-StudySIOP/HIT LGG-Study• study for therapy optimization by
randomized testing of two treatment concepts for children and adolescents with low-grade gliomas
• approximately 800 patients from german speaking countries at the date of evaluation (3/07)
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• 149 patients with optic pathway glioma
• 143 patients evaluated
–44 NF1+
–99 NF1-• Evaluation of MRI at time of diagnosis
Study patientsStudy patientsStudy patientsStudy patients
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Demographic DataDemographic DataDemographic DataDemographic Data
NF1- NF1+number 99 44
age (Median) 4.13 y 5.25 y
patients
<1 y/o16 (16%) 0
gender49% f
51% m44% f
56% m
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Features of interestFeatures of interest• Affected anatomic structures
• Growth form
• Further CNS tumours
• Menigeal tumour spread
• Gliomatosis cerebri
• Contrast enhancement
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Detailed Anatomic DescriptionDetailed Anatomic Description
Affected anatomic structures:• optic nerve
-intraorbital-extraorbital
•optic chiasm•hypothalamus•optic tract•optic radiation•CNS outside optic pathway
Classifications:
• Dodge Classification:
• Dodge I - III
• PLAN Classification
(not yet employed)
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AnatomicAnatomic LocalisationLocalisationNF1- NF1+
solely optic nerve
unilateral 6/99
bilateral 5/99
unilateral 6/44
bilateral 2/44
solely chiasm 52/99 1/44
chiasm and postchiasmatic optic pathway
16/99 3/44
optic nerve, chiasm und postchiasmat. optic pathway
17/99 28/44
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Optic nerveOptic nerveNF1- NF1+
intraorbital 19% 70%
extraorbital 20% 80%
both 9% 63%
optic nerve not involved
69% 11%
optic chiasm not involved
11% 20%
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Chiasma and beyondChiasma and beyond
NF1- NF1+optic chiasm 89% 80%
hypothalamus 66% 70%
optic tract 34% 67%
optic radiation 2% 22%
CNS outside optic pathway
3% 17%
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Form of tumour growthForm of tumour growthForm of tumour growthForm of tumour growthNF1- NF1+
focal 72% 23%
diffuse 28% 73%
combination 1% 5%
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focal vs. diffusefocal vs. diffusefocal vs. diffusefocal vs. diffuse
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Special CharacteristicsSpecial CharacteristicsSpecial CharacteristicsSpecial Characteristics
NF1- NF1+meningeal tumour spread
8% 0%
further CNS tumours
0% 18%
Gliomatosis 0% 7%
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Meningeal Tumour SpreadMeningeal Tumour SpreadMeningeal Tumour SpreadMeningeal Tumour Spread
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Second Second Tumour and Gliomatosis Tumour and Gliomatosis
Second Second Tumour and Gliomatosis Tumour and Gliomatosis
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Enhancement of contrast Enhancement of contrast agentagent
Enhancement of contrast Enhancement of contrast agentagent
NF1- NF1+
Enhancement 93% 76%
none 7% 24%
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SummarySummarySummarySummary• typical of NF1-: isolated affection of chiasm
• typical of NF1+: widespread, diffuse affection of optic nerve, chiasm and postchiasmatic optic pathway
• Further CNS tumours or criteria of Gliomatosis solely in NF1+
• Meningeal tumour spread not in NF1+ patients at time of diagnosis
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Thank you!