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

4. Demographic Data NF1- NF1+ number 99 44 age (Median) 4.13 y 5.25 y
patients
<1 y/o
16 (16%)
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 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

8. Optic nerve NF1- NF1+ intraorbital 19% 70% extraorbital 20% 80% both
63%
optic nerve not involved
69%
11%
optic chiasm not involved

9. Chiasma and beyond NF1- NF1+ optic chiasm hypothalamus optic tract
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+ focal 72% 23% diffuse 28% 73%
combination 1% 5%

11. focal vs. diffuse

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

13. Meningeal Tumour Spread

14. Second Tumour and Gliomatosis

15. Enhancement of contrast agent
NF1-
NF1+
Enhancement
93%
76%
none 7%
24%

16. Summary 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!