Current and emerging treatment strategies for children with progressive chiasmatic-hypothalamic glioma diagnosed as infants: a web-based survey

Amedeo A Azizi,Antoinette Y N Schouten-Van Meeteren

doi:10.1007/s11060-017-2630-6

Amedeo A Azizi, Antoinette Y N Schouten-Van Meeteren

Open Access

https://doi.org/10.1007/s11060-017-2630-6

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Abstract

Treatment of infant hypothalamic chiasmatic glioma (iCHG) is challenging, about 30% of the children progress during chemotherapy. Despite subsequent treatments the 5 year overall-survival rate is only 70%. This study investigates treatment strategies currently applied for progressive iCHG. A web-based questionnaire was sent out to the members of the SIOPE Brain Tumour Group asking for current second and third line strategies at progression during and after the end of first line therapy. The questionnaire was answered by 47 paediatric oncologists from 15 countries. iCHG progressing during first line therapy with carboplatin-vincristine would be considered for treatment with alternative chemotherapy by 17 (36%) and with surgery plus chemotherapy by 27 respondents (58%). Components suggested for second line were vinblastine (62%), cisplatin (34%) and cyclophosphamide (26%). For third line therapy bevacizumab (BVZ) was considered as suitable by respondents in 53% (often with irinotecan 40%) and vinblastine by 34% respectively. Experience with BVZ in CHG is shown by 53% of respondents regarding at least 95 patients (median treated 1–5 patients per respondent at any age) with a median BVZ administration over 12 months. Effectiveness was reported varying between stable disease and regression while complications were rarely stated (proteinuria, hypertension, bleeding). BVZ would be available to 85% of respondents as therapeutic option for iCHG patients. Multiple anti-neoplastic drug regimens are applied for progressive iCHG, partly considered in combination with surgery if safely feasible. BVZ is commonly used at a satisfactory level in third line, mainly combined with irinotecan.

Highlights

Low-grade gliomas (LGG) are the most common brain tumours in childhood, accounting for approximately 35%during the first year of life and up to 50% in older children [1]
Additional questions were asked on characteristics of the participating medical specialist: profession, country of origin, years of clinical experience, yearly amount of children treated with a brain tumour and the subgroups of LGG, CHG as well as infant chiasmatic hypothalamic glioma (iCHG)
Multiple different drug regimens are applied for progressive iCHG

Summary

Introduction

Low-grade gliomas (LGG) are the most common brain tumours in childhood, accounting for approximately 35%during the first year of life and up to 50% in older children [1]. The chiasmatic/hypothalamic region is the predilective location in infants, and 20% of all intracranial tumours in children below the age of 2 years occur in this region [2]. The survey took around 15 min to fill in and consisted of questions regarding treatment strategies, drugs applied for systemic therapy and experience with bevacizumab in CHG patients. Additional questions were asked on characteristics of the participating medical specialist: profession, country of origin, years of clinical experience, yearly amount of children treated with a brain tumour and the subgroups of LGG, CHG as well as iCHG . As the survey aimed at assessing general treatment strategies, it did not collect data on patient characteristics (e.g. age at relapse, pattern of relapse or initial symptoms such as diencephalic syndrome)

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