合并间脑综合征的儿童视路胶质瘤化疗后体重增长及生存观察

doi:10.12372/jcp.2024.23e0869

摘要/Abstract

摘要：

目的探讨伴有间脑综合征（DS）的视路胶质瘤（OPG）患儿化疗后的体重增长及生存状况。方法回顾性分析2018年5月—2020年12月儿科收治的6例伴有DS的OPG患儿临床资料。患儿确诊中位年龄13.5（3~43）个月，化疗前中位体重为6.9（6~8）kg，所有患儿在确诊OPG之前均出现消瘦或体重不增，化疗前均呈极度消瘦状态，其年龄别体重Z评分（WAZ）均<-2。化疗过程中动态监测患儿的体重变化，评估化疗后1、3、6、9、12个月的WAZ，采用重复测量方差分析比较患儿化疗前后WAZ并对其生存状况进行观察随访。结果中位随访时间46（29~59）个月，2例视力消失的患儿，经手术或放化疗后视力仍无明显改善，4例患儿起病时伴有眼震，化疗后眼震均消失，1例患儿出现中枢性性早熟。结束化疗后1例失访，3例停止化疗后肿瘤进展，予化疗维持肿瘤稳定，无死亡病例。所有患儿体重较化疗前明显增长，随着化疗时间延长，体重及WAZ增加明显（P<0.05）。结论伴有DS的儿童OPG，术后化疗有助于改善眼震、增加体重。

关键词: 间脑综合征, 视路胶质瘤, 儿童, 体重增长

Abstract:

Objective To investigate the ponderal growth and survival post-chemotherapy of optic pathway glioma (OPG) in children with diencephalon syndrome (DS). Methods Six OPG patients with DS admitted from May 2018 to December 2020 were analyzed retrospectively. The median age of children was 13.5 (3 - 43) months. The median weight Pre-chemotherapy of children was 6.9(6-8) kg. None of the children gained weight prior to the OPG diagnosis, and all exhibited cachexia and extreme emaciation upon admission. The weight-for-age Z score (WAZ) was below -2 in all six patients pre-chemotherapy. The weight changes were dynamically monitored during chemotherapy, and WAZ was assessed at 1, 3, 6, 9 and 12 months post-chemotherapy. Repeated measures analysis of variance was used to compare the WAZ and survival at different time points after chemotherapy. Results The median follow-up time was 46(29-59) months. Visual acuity did not significantly improve in two children with anopsia after surgery or chemo-radiotherapy. Nystagmus, present in four patients at disease onset, resolved post-chemotherapy and one child had central precocity. After chemotherapy, one patient was lost to follow-up, and three experienced tumor progression, which was managed with maintenance chemotherapy to stabilize the disease. There were no fatalities.The weight of all children increased significantly compared with that prechemotherapy, and WAZ increased significantly with the extension of chemotherapy time (P<0.05). Conclusion In children with DS with OPG, postoperative chemotherapy can improve ocular symptoms and promote weight gain.

Key words: diencephalon syndrome, optic pathway glioma, child, ponderal growth

刘晶晶, 武万水, 杜淑旭, 孙艳玲, 龚小军, 张金, 李苗, 孙黎明. 合并间脑综合征的儿童视路胶质瘤化疗后体重增长及生存观察[J]. 临床儿科杂志, 2024, 42(10): 876-880.

LIU Jingjing, WU Wanshui, DU Shuxu, SUN Yanling, GONG Xiaojun, ZHANG Jin, LI Miao, SUN Liming. Ponderal growth and survival post-chemotherapy of optic pathway glioma in children with diencephalon syndrome[J]. Journal of Clinical Pediatrics, 2024, 42(10): 876-880.

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患儿	月龄	性别	起病症状	病理类型	NF-1突变	BRAF突变	治疗方案	化疗前体重/kg	化疗前WAZ
例1	43	女	消瘦、视力消失	PA	未测	未测	化疗、放疗	8	-4.87
例2	15	男	眼震、消瘦	PMA	阴性	阴性	化疗	8	-2.82
例3	10	男	消瘦、眼震	PA	阴性	BRAF融合	化疗	6	-4.95
例4	3	女	呕吐、体重不增	PMA	阴性	阴性	化疗	6.3	-3.93
例5	12	男	消瘦、眼震	PA	阴性	阴性	化疗	7.5	-3.32
例6	18	男	消瘦、眼震、视力消失	间变星形	阴性	阴性	化疗	6.3	-4.89

患儿	化疗前后Z评分变化	进展	PFS	生存结局	OS
例1	5.16	否	3个月	存活	58个月
例2	2.96	是	30个月	存活	62个月
例3	4.54	是	34个月	存活	63个月
例4	4.38	失访	-	失访	-
例5	3.71	是	31个月	存活	34个月
例6	3.13	否	31个月	存活	31个月