gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

Comprehensive treatment strategy for childhood visual pathway glioma

Meeting Abstract

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  • corresponding author A. K. Gnekow - I. Hospital for Children and Adolescents, Klinikum Augsburg - for the study committee of the low grade glioma study, working group on brain tumors of the Society for Pediatric Oncology and Hematology (GPOH)

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogSA.02.02

The electronic version of this article is the complete one and can be found online at:

Published: September 22, 2004

© 2004 Gnekow.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Low grade glioma of all age groups and CNS localization represent 30 - 40% of brain tumors in children and adolescents. Their growth capacity cannot be predicted by present criteria. Tumor resection is the treatment of choice, the extent of resection is limited, however, by the risk of inacceptable post-surgical morbidity. The significant risk of progression for incompletely resected tumors requires a strategy for the use of non-surgical therapeutic modalities. Late effects of radiotherapy, especially concerning psychointellectual development have prompted the use of chemotherapy, especially in young children with chiasmatic-hypothalamic tumors. Within the HIT-LGG 1996 study strategy early radiotherapy shall be avoided by chemotherapy.

22 of 905 protocol patients (2,4%) had an isolated tumor of the optic nerve, 13/24 associated with NF I. Following observation (11) or chemotherapy (10) or radiotherapy (1) only 1 tumor is persistently progressive.

198 children (21,9%) had a chiasmatic (34), chiasmatic-hypothalamic (144) or hypothalamic (20) primary at a median age of 3,6 years (0,2 - 16,3 years), 56 had Neurofibromatosis NF I (28,3%), 107 female (54%). 92 children had severe visual impairment as primary symptom. The extent of neurosurgical resection was small, in 55 children diagnosis was based upon neuroradiological findings. Besides 133 pilocytic astrocytoma °I, 5 astrocytoma °II/nos and 2 DIGG/DIA °I were histologically verified (3 not known).

82 children were treated at diagnosis, 68 upon clinical or radiological progression following observation times of 3,1 to 116,6 months.

Radiotherapy: 27 received a conventional (18) or interstitial (8) radiotherapy (1 not known) at a median age of 7,3 years. At a median follow-up of 50,1 months 21 tumors are stable, 3 regressive (2 not evaluable, 1 child died).

Chemotherapy: 123 children received Vincristin/Carboplatin at a median age of 3,7 years. 105/123 achieved a CR/PR/SD, 44/123 tumors were progressive following a median time of 22,8 months. 16/44 children were later irradiated and had reached a median age of 7,3 years at this point. Following an observation time of median 44,7 months 93 tumors are stable, 10 regressive, 9 progressive. 4 children died, 7 are not evaluable.

At 60 months overall survival of the entire group is 0,93; progression free survival is 0,72 for the radiotherapy group and 0,61 for the chemotherapy group, their radiotherapy-free survival is 0,83. Within the chemotherapy group an age at diagnosis < 1 year or non-pilocytic histology carry an increased risk of progression.

For the European study SIOP-LGG 2004 uniform neurologic, ophthalmologic and radiologic criteria have been defined for the indication for non-surgical therapy. They apply for chemo- as well as for radiotherapy and distinguish between the start of therapy at diagnosis and following observation. The possibility of a more effective reduction of the risk of progression by intensifying chemotherapy shall be investigated in a randomized trial. Since efficacy of this strategy is measured by the possibility to maintain an adequate vision for children with visual pathway glioma, the results of age-adapted follow-up investigations have to be documented in a consequent manner.