MR- Morphology and exact Anatomic Localisation of Optic Pathway Gliomas in children with and without...
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Transcript of MR- Morphology and exact Anatomic Localisation of Optic Pathway Gliomas in children with and without...
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
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)
• 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
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
Features of interestFeatures of interest• Affected anatomic structures
• Growth form
• Further CNS tumours
• Menigeal tumour spread
• Gliomatosis cerebri
• Contrast enhancement
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)
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
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%
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%
Form of tumour growthForm of tumour growthForm of tumour growthForm of tumour growthNF1- NF1+
focal 72% 23%
diffuse 28% 73%
combination 1% 5%
focal vs. diffusefocal vs. diffusefocal vs. diffusefocal vs. diffuse
Special CharacteristicsSpecial CharacteristicsSpecial CharacteristicsSpecial Characteristics
NF1- NF1+meningeal tumour spread
8% 0%
further CNS tumours
0% 18%
Gliomatosis 0% 7%
Meningeal Tumour SpreadMeningeal Tumour SpreadMeningeal Tumour SpreadMeningeal Tumour Spread
Second Second Tumour and Gliomatosis Tumour and Gliomatosis
Second Second Tumour and Gliomatosis Tumour and Gliomatosis
Enhancement of contrast Enhancement of contrast agentagent
Enhancement of contrast Enhancement of contrast agentagent
NF1- NF1+
Enhancement 93% 76%
none 7% 24%
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
Thank you!