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11号染色体杂合性缺失状态对伴有MYCN扩增的高危神经母细胞瘤新辅助化疗反应的影响

Impact of 11q Loss of Heterozygosity Status on the Response of High-Risk Neuroblastoma With MYCN Amplification to Neoadjuvant Chemotherapy.

作者信息

Lu Xian-Ying, Qu Li-Jun, Duan Xian-Lun, Zuo Wei, Sai Kai, Rui Gang, Gong Xian-Feng, Ding Yi-Bo, Gao Qun

机构信息

Department of General Surgery, Anhui Children's Hospital, Hefei, China.

Department of Hematology and Oncology, Anhui Children's Hospital, Hefei, China.

出版信息

Front Pediatr. 2022 Jun 10;10:898918. doi: 10.3389/fped.2022.898918. eCollection 2022.

Abstract

PURPOSE

The aim of this study was to investigate whether 11q loss of heterozygosity (LOH) aberration would impact the response of the primary tumor to neoadjuvant chemotherapy or to the degree of surgical resection in neuroblastoma (NB) patients with MYCN amplification.

METHODS

The clinical data of 42 NB patients with MYCN amplification who were newly diagnosed and received treatments at our hospital from 2011 to 2020 were retrospectively analyzed. According to the results of the segmental chromosome aberration analysis, the patients enrolled were assigned to an 11qLOH positive group and an 11qLOH negative group.

RESULTS

There was no significant difference in the mean number of chemotherapy courses completed before surgery between the 11qLOH positive and 11qLOH negative groups ( = 0.242). Each of the 42 patients had metaiodobenzylguanidine (MIBG) scans both before and after neoadjuvant chemotherapy. The percentage of patients who had a clinical MIBG change in the 11qLOH positive group was lower than the percentage in the 11qLOH negative group (27.27 vs. 66.67%, = 0.030). The 11qLOH negative group seemed to have a higher rate of surgical resection (≥90%); however, the difference between the two groups was not statistically significant ( = 0.088). Furthermore, the 11qLOH negative group did not show significantly superior event-free survival and overall survival rates compared with the 11qLOH positive group.

CONCLUSIONS

This study showed that patients with NB and MYCN amplification in combination with 11qLOH might be less likely to respond to neoadjuvant chemotherapy when compared with patients with NB and MYCN amplification without 11qLOH.

摘要

目的

本研究旨在调查11号染色体杂合性缺失(LOH)畸变是否会影响MYCN基因扩增的神经母细胞瘤(NB)患者的原发肿瘤对新辅助化疗的反应或手术切除程度。

方法

回顾性分析2011年至2020年在我院新诊断并接受治疗的42例MYCN基因扩增的NB患者的临床资料。根据节段性染色体畸变分析结果,将入组患者分为11qLOH阳性组和11qLOH阴性组。

结果

11qLOH阳性组和11qLOH阴性组术前完成化疗疗程的平均数量无显著差异( = 0.242)。42例患者在新辅助化疗前后均进行了间碘苄胍(MIBG)扫描。11qLOH阳性组临床MIBG改变的患者百分比低于11qLOH阴性组(27.27%对66.67%, = 0.030)。11qLOH阴性组似乎有更高的手术切除率(≥90%);然而,两组之间的差异无统计学意义( = 0.088)。此外,与11qLOH阳性组相比,11qLOH阴性组的无事件生存率和总生存率并未显示出显著优势。

结论

本研究表明,与无11qLOH的MYCN基因扩增的NB患者相比,伴有11qLOH的MYCN基因扩增的NB患者对新辅助化疗的反应可能较小。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/439a/9226623/36c26f91ec5c/fped-10-898918-g0001.jpg

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