Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Spitalgasse 23, 1090, Vienna, Austria.
Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.
J Neurooncol. 2018 Jan;136(1):127-134. doi: 10.1007/s11060-017-2630-6. Epub 2017 Oct 25.
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.
婴儿下丘脑视交叉神经胶质瘤（iCHG）的治疗具有挑战性，约 30%的患儿在化疗过程中病情进展。尽管随后进行了治疗，但 5 年总生存率仅为 70%。本研究调查了目前用于进展性 iCHG 的治疗策略。我们向 SIOPE 脑肿瘤组的成员发送了一份基于网络的问卷，询问他们在一线治疗结束后进展期间目前的二线和三线治疗策略。来自 15 个国家的 47 名儿科肿瘤学家回答了这个问卷。17 名（36%）受访者认为，在一线治疗中使用卡铂-长春新碱治疗后病情进展的 iCHG 可考虑改用其他化疗药物，27 名（58%）受访者建议手术加化疗。二线治疗建议使用长春碱（62%）、顺铂（34%）和环磷酰胺（26%）。53%的受访者认为贝伐单抗（BVZ）适合作为三线治疗药物（常与伊立替康 40%联合使用），34%的受访者认为长春碱适合作为三线治疗药物。53%的受访者表示他们在 CHG 方面有使用 BVZ 的经验，涉及至少 95 名患者（中位数为每位患者在任何年龄治疗 1-5 例），中位 BVZ 给药时间超过 12 个月。疗效报告显示为疾病稳定和消退，而并发症很少被提及（蛋白尿、高血压、出血）。85%的受访者将 BVZ 作为 iCHG 患者的治疗选择。对于进展性 iCHG，会应用多种抗肿瘤药物治疗方案，部分方案考虑与手术联合应用，如果安全可行的话。BVZ 通常在三线治疗中得到广泛应用，主要与伊立替康联合使用。
