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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.

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Presentation on theme: "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."— Presentation transcript:

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 *, L. Solymosi², M. Warmuth-Metz² ¹Klinik für Radiologische Diagnostik, RWTH Aachen, ²Abt. für Neuroradiologie, Universität Würzburg, * SIOP/HIT-LGG Studienzentrale Augsburg

2 SIOP/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)

3 149 patients with optic pathway glioma 143 patients evaluated –44 NF1+ –99 NF1- Evaluation of MRI at time of diagnosis Study patients

4 Demographic Data NF1-NF1+ number9944 age (Median) 4.13 y5.25 y patients <1 y/o 16 (16%)0 gender 49% f 51% m 44% f 56% m

5 Features of interest Affected anatomic structures Growth form Further CNS tumours Menigeal tumour spread Gliomatosis cerebri Contrast enhancement

6 Detailed 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)

7 Anatomic Localisation NF1-NF1+ solely optic nerve unilateral 6/99 bilateral 5/99 unilateral 6/44 bilateral 2/44 solely chiasm52/991/44 chiasm and postchiasmatic optic pathway 16/993/44 optic nerve, chiasm und postchiasmat. optic pathway 17/9928/44

8 Optic nerve NF1-NF1+ intraorbital19%70% extraorbital20%80% both9%63% optic nerve not involved 69%11% optic chiasm not involved 11%20%

9 Chiasma 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%

10 Form of tumour growth NF1-NF1+ focal72%23% diffuse28%73% combination1%5%

11 focal vs. diffuse

12 Special Characteristics NF1-NF1+ meningeal tumour spread 8%0% further CNS tumours 0%18% Gliomatosis0%7%

13 Meningeal Tumour Spread

14 Second Tumour and Gliomatosis

15 Enhancement of contrast agent NF1-NF1+ Enhancement93%76% none7%24%

16 SummarySummary 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

17 Thank you!


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