G4型髓母细胞瘤患儿预后影响因素及生存状况分析

doi:10.12372/jcp.2023.22e1634

摘要/Abstract

摘要：

目的探讨儿童G4型髓母细胞瘤（MB）患者的生存状况及其预后影响因素。方法回顾性分析2016年5月至2020年8月儿科收治的G4型MB患儿（随访至2022年8月）的临床资料，采用Kaplan-Meier法计算总体生存（OS）率和无进展生存（PFS）率，采用Log-rank检验比较组间生存率差异，采用Cox回归模型分析影响预后的因素。结果纳入145例G4型MB患儿，男106例、女39例，中位确诊年龄7.5（5.7~9.6）岁。M₀期91例，M₊期54例（M₁期1例、M₂期12例、M₃期41例）;病理分型为经典型127例、促纤维增生/结节型（DN）8例、大细胞/间变型（LC/A）8例、广泛结节型（EN）1例、未分型1例。中位随访时间为47.9（36.5~59.3）月，复发37例。患儿5年OS率和PFS率分别为（80.8±3.4）%和（55.4±4.7）%。Cox回归分析结果提示M₊期、MYCN扩增、Chr12 p+变异是影响预后的独立危险因素（P<0.05）。结论 M₊期、MYCN扩增的G4型MB患儿预后相对较差。Chr12 p+可能与患儿预后相关，但仍有待更大样本的临床研究进行证实。

关键词: G4型髓母细胞瘤, 预后, 影响因素, 儿童

Abstract:

Objective To investigate the survival status and prognostic factors of group 4 medulloblastoma (MB) in children. Methods The clinical data of children with group 4 MB admitted to the Department of Pediatrics from May 2016 to August 2020 (follow-up until August 2022) were retrospectively analyzed. The Kaplan-Meier method was used to calculate the overall survival (OS) rate and progression-free survival (PFS) rate. Log-rank test was used to compare the difference in survival rate between groups, and Cox regression model was used to analyze the factors affecting prognosis. Results A total of 145 children (106 boys and 39 girls) with group 4 MB were included, and the median age of diagnosis was 7.5 (5.7-9.6) years old. There were 91 children in M₀ stage and 54 children in M₊ stage (1 in M₁ stage, 12 in M₂ stage and 41 in M₃ stage). The pathologic types were classic in 127 cases, desmoplastic/nodular (DN) in 8 cases, anaplastic/large cell (LC/A) in 8 cases, extensive nodularity (EN) in 1 case, and none of somatotype (NOS) in 1 case. The median follow-up time was 47.9 (36.5-59.3) months, and 37 children had tumor recurrence. The 5-year OS and PFS rates were (80.8±3.4) % and (55.4±4.7) %, respectively. Cox regression analysis indicated that M₊ stage, MYCN amplification and Chr12p+ variation were independent risk factors for prognosis (P<0.05). Conclusions Group 4 MB children with M₊ stage or MYCN amplification have a relatively poor prognosis. Chr12p + may be related to the prognosis of children, but it needs to be confirmed by clinical studies with larger samples.

Key words: group 4 medulloblastoma, prognosis, influencing factor, child

武跃芳, 孙艳玲, 武万水, 杜淑旭, 李苗, 孙黎明. G4型髓母细胞瘤患儿预后影响因素及生存状况分析[J]. 临床儿科杂志, 2023, 41(9): 686-691.

WU Yuefang, SUN Yanling, WU Wanshui, DU Shuxu, LI Miao, SUN Liming. Analysis of prognostic factors and survival status of group 4 medulloblastoma in children[J]. Journal of Clinical Pediatrics, 2023, 41(9): 686-691.

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项目	例数	3年OS率/%	χ²值	P	3年PFS率/%	χ²值	P
性别			0.79	0.376		1.63	0.201
男	106	87.7±3.2			60.9±4.8
女	39	82.1±6.1			71.7±7.2
手术范围			0.02	0.882		1.43	0.233
GTR	103	87.3±3.3			61.7±4.8
NTR/STR	42	83.3±5.8			68.9±7.2
分期			10.96	0.001		13.02	<0.001
M₀	91	92.3±2.8			74.0±4.7
M₊	54	75.9±5.8			47.3±6.7
病理分型			0.85	0.932		2.33	0.676
CMB	127	86.6±3.0			61.8±4.3
DN	8	87.5±11.7			87.5±11.7
LC/A	8	75.0±15.3			62.5±17.1
EN	1	-			-
NOS	1	-			-
染色体变异
有MYCN+	11	72.7±13.4	6.26	0.012	36.4±14.5	7.48	0.006
无MYCN+	134	87.3±2.9	6.26	0.012	66.2±4.1	7.48	0.006
有Chr2p+	6	83.3±15.2	1.18	0.277	50.0±20.4	0.32	0.572
无Chr2p+	139	86.3±2.9	1.18	0.277	64.5±4.1	0.32	0.572
有Chr12p+	7	57.1±18.7	4.12	0.042	14.3±13.2	12.55	<0.001
无Chr12p+	138	87.7±2.8	4.12	0.042	66.4±4.0	12.55	<0.001
有Chr17p+	15	100.0±0.0	3.41	0.065	80.0±10.3	2.98	0.085
无Chr17p+	130	84.6±3.2	3.41	0.065	62.0±4.3	2.98	0.085
有Chr17q+	72	87.5±3.9	0.60	0.440	58.9±5.9	0.88	0.350
无Chr17q+	73	84.9±4.2	0.60	0.440	68.5±5.4	0.88	0.350
有Chr7q+	65	81.5±4.8	2.12	0.145	51.8±6.3	7.06	0.008
无Chr7q+	80	90.0±3.4	2.12	0.145	73.5±5.0	7.06	0.008
有Chr7+	67	82.1±4.7	1.67	0.197	53.3±6.2	5.64	0.018
无Chr7+	78	89.7±3.4	1.67	0.197	72.8±5.1	5.64	0.018
有Chr17+	74	87.8±3.8	0.87	0.350	58.6±5.8	0.98	0.323
无Chr17+	71	84.5±4.3	0.87	0.350	69.0±5.5	0.98	0.323
有Chr18+	16	81.3±9.8	0.02	0.893	68.2±11.8	0.50	0.479
无Chr18+	129	86.8±3.0	0.02	0.893	63.3±4.3	0.50	0.479
有Chr8p-	7	87.5±8.3	0.00	0.962	50.0±12.5	0.89	0.347
无Chr8p-	138	86.0±3.1	0.00	0.962	65.6±4.2	0.89	0.347
有Chr8q-	16	85.7±13.2	0.07	0.795	57.1±18.7	0.11	0.742
无Chr8q-	129	86.2±2.9	0.07	0.795	64.2±4.1	0.11	0.742
有Chr11-	9	77.8±13.9	0.06	0.815	77.8±13.9	1.20	0.274
无Chr11-	136	86.7±2.9	0.06	0.815	62.9±4.2	1.20	0.274
有Chr8-	15	86.7±8.8	0.03	0.853	46.7±12.9	1.46	0.227
无Chr8-	130	86.1±3.0	0.03	0.853	65.9±4.2	1.46	0.227

因素	PFS			OS
因素	HR	95%CI	P	HR	95%CI	P
M₊期	2.89	1.71~4.87	<0.001	4.06	1.77~9.33	0.001
MYCN扩增	3.73	1.79~7.79	<0.001	5.14	1.85~14.28	0.002
Chr12p+	5.28	2.20~12.70	<0.001	3.78	1.09~13.07	0.036