高危神经母细胞瘤儿童的临床特点和生存状况研究
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湖南省儿童医院

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2021年度湖南省卫生健康委科研立项课题(项目编号:202104081062)


Study on the clinical characteristics and survival status of children with high-risk neuroblastoma
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    摘要:

    目的 分析高危神经母细胞瘤儿童的临床特点和生存状况。 方法 选取2016年1月-2020年1月期间我院收治的83例高危神经母细胞瘤儿童为研究对象,回顾性分析高危神经母细胞瘤儿童的临床特征和生存状况,对高危神经母细胞瘤儿童死亡的相关因素进行单因素和Logistic多因素分析。 结果 高危神经母细胞瘤儿童的主要发病人群为18-60月龄儿童,男性居多,肾上腺或腹膜后为主要发病部位,多数患儿伴随骨髓转移、骨骼转移、远处淋巴结转移,肾上腺或腹膜后<1500U/L、NSE为正常值至100ng/ml、尿VMA阴性、血红蛋白<90g/L、MYCN未扩增,临床表现以发热、咳嗽、查体(无症状)、腹痛/腹胀、贫血、骨关节疼痛为主。对83例患儿随访18-78个月,共死亡50例(60.24%),失访3例(3.61%),存活30例(36.15%),发生不良事件59例(71.08%),3年EFS为38.55%、5年EFS为31.33%,3年OS为49.40%,5年OS为36.15%。单因素分析显示,骨髓转移、骨骼转移、诊断时LDH≥1500U/L、尿VMA阴性、血红蛋白>110g/L、MYCN扩增、NSE缓解时间≤2个疗程、骨髓缓解速度>2个疗程、国际神经母细胞瘤临床分期(INSS)为Ⅲ-Ⅳ期、部分切除病灶的高危神经母细胞瘤儿童5年OS较短,差异统计学有意义(p<0.05)。Logistic多因素分析显示,骨髓转移、诊断时LDH≥1500U/L、MYCN扩增、INSS分期为Ⅲ-Ⅳ期、部分切除病灶为高危神经母细胞瘤儿童死亡的危险因素(p<0.05)。 结论 高危神经母细胞瘤儿童表现出明显的临床特点,可帮助临床诊断,患儿5年生存率不足40%,其中骨髓转移、诊断时LDH、MYCN扩增、INSS分期、部分切除病灶为患儿死亡的危险因素,应积极给予干预。

    Abstract:

    Objective To analyze the clinical characteristics and survival status of children with high-risk neuroblastoma. Methods A total of 83 children with high-risk neuroblastoma treated in our hospital from January 2016 to January 2020 were selected as the research subjects. The clinical characteristics and survival status of children with high-risk neuroblastoma were retrospectively analyzed. The related factors of child death were analyzed by univariate and Logistic multivariate analysis. Results The main incidence of children with high-risk neuroblastoma was 18-60 months of age, mostly males. The adrenal gland or retroperitoneum were the main sites of incidence. Most children were accompanied by bone marrow metastasis, bone metastasis, distant lymph node metastasis, adrenal gland or retroperitoneum. <1500U/L, NSE is normal to 100ng/ml, urine VMA is negative, hemoglobin <90g/L, MYCN is not amplified, clinical manifestations include fever, cough, physical examination (asymptomatic), abdominal pain/bloating, anemia, bone Joint pain is predominant. Follow-up of 83 children for 18-78 months, 50 cases died (60.24%), 3 cases were lost to follow-up (3.61%), 30 cases survived (36.15%), 59 cases (71.08%) occurred adverse events, 3 years The EFS was 38.55%, the 5-year EFS was 31.33%, the 3-year OS was 49.40%, and the 5-year OS was 36.15%. Univariate analysis showed that bone marrow metastasis, bone metastasis, LDH≥1500U/L at diagnosis, urinary VMA negative, hemoglobin>110g/L, MYCN amplification, NSE remission time ≤ 2 courses, bone marrow remission rate> 2 courses, international The clinical staging of neuroblastoma (INSS) was Ⅲ-Ⅳ, and the 5-year OS of children with high-risk neuroblastoma with partial resection of the lesion was shorter, and the difference was statistically significant (p<0.05). Logistic multivariate analysis showed that bone marrow metastasis, LDH≥1500U/L at diagnosis, MYCN expansion, INSS stage III-IV, and partial resection of lesions were risk factors for high-risk neuroblastoma death in children (p<0.05). Conclusion Children with high-risk neuroblastoma show obvious clinical features, which can help clinical diagnosis. The 5-year survival rate of children is less than 40%. Among them, bone marrow metastasis, LDH, MYCN amplification, INSS staging, and partial resection of the lesion are children with bone marrow metastasis, LDH at diagnosis, and partial resection of the lesion. The risk factors of death should be actively intervened.

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  • 收稿日期:2021-06-18
  • 最后修改日期:2021-06-18
  • 录用日期:2021-08-18
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