Aims The natural history of children with NF1-OPG is unpredictable, indications for therapy to save vision, using patient, imaging and visual factors have not been studied in Europe. In the previous SIOP-E NF1-OPG multi-disciplinary workshop, consensus on imaging and visual classification was achieved, including a schematic for recording patient, visual and imaging details. This survey was setup to collect opinions on selecting NF1-associated OPG patients for observation, treatment and randomisation, and assess the feasibility of a randomised trial from an external multidisciplinary group.

Methods Eight SIOP-LGG2004 centres contributed 83 NF1-OPG cases with complete imaging, visual and clinical datasets. From these, 10 cases were selected to display a range of ages on presentation and tumour locations in accordance with the PLAN classification (Figure 1).¹

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Abstract G14 Figure 1

PLAN classificatioin¹, visual acuity and age at diagnosis of the ten patients selected for the survey

The survey targets all medical professionals involved in the multi-disciplinary team making management decisions for NF1-OPG patients. The questionnaire consists of 32 questions to collect data on (1) management decision of 10 representative cases and reasoning, (2) information about the participant and (3) feedback on the questionnaire. This questionnaire was pre-tested among SIOP-E NF1-OPG workshop participants in late 2014 then revised in 2015.

Results Sixty-two medical professionals took part in the survey and allocated 4 cases for treatment and 2 cases for observation with consensus (>70% agreement). Respondents did not reach consensus on the management decision of 4 patients (Table 1); 20–43% of the respondents voted for randomisation for the four patients.

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Abstract G14 Table 1

Survey results from 62 medical professionals involved in the multi-disciplinary team making management decisions for the ten NF1-OPG patients

Conclusions The SIOP-E NF1-OPG group will invite more medical professionals to express their opinion on using patient, imaging and visual factors to select patients for sight-saving therapy. The results will assist the group in planning the next SIOP-LGG trial. Subgroups that did not reach consensus on treatment decision may be eligible for randomisation.

Reference

1. Taylor T, Jaspan T, Milano G, et al. Radiological classification of optic pathway gliomas: experience of a modified functional classification system. The British Journal of Radiology 2008;81(970):761–6