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.

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!