Fan Hongjun, Xing Tianyu, Hong Huimin, Duan Chao, Zhao Wen, Zhao Qian, Wang Xisi, Huang Cheng, Zhu Shuai, Jin Mei, Su Yan, Gao Chao, Ma Xiaoli
Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Ocology, Key Laboratory of Major Diseases in Children, Ministry of Education, 56 Nan Lishi Road, Xicheng District, Beijing, China.
Hematologic Disease Laboratory, Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, China.
Pediatr Hematol Oncol. 2022 May;39(4):343-356. doi: 10.1080/08880018.2021.1995090. Epub 2021 Nov 9.
Paired-like homeobox 2B () is a highly sensitive and specific biomarker for diagnosing neuroblastoma, as well as detecting minimal residual disease in neuroblastoma. The clinical significance of expression in bone marrow (BM) and peripheral blood (PB) samples of newly diagnosed patients with very low-, low- and intermediate-risk neuroblastoma remains unknown, to the best of our knowledge. The expression level of in paired BM and PB samples of patients with newly diagnosed neuroblastoma was validated using reverse transcription-quantitative polymerase chain reaction (RTqPCR). Among the 132 patients, 26 exhibited a positive expression BM (19.7%) and 11 in PB (8.3%) samples. was highly expressed in BM and PB samples from patients aged <18 months, with International Neuroblastoma Risk Group Staging System stages M and MS, 1p loss of heterozygosity, and high levels of lactate dehydrogenase, serum ferritin and neuron-specific enolase ( < 0.05). In all eligible patients, the 2-year event-free survival (EFS) and overall survival (OS) rates were 94.7 ± 2.0% and 97.7 ± 1.3%, respectively. However, the 2-year EFS rates were significantly decreased to 76.9 ± 8.3% and 63.6 ± 14.5% in patients with a positive expression in BM and PB samples, respectively ( < 0.05). Similarly, the 2-year OS rates were also decreased to 88.5 ± 6.3% and 81.8 ± 11.6% in patients with a positive expression in BM and PB samples, respectively ( < 0.05). In conclusion, a positive expression in BM and PB samples at diagnosis had a strong adverse prognostic effect on patients with non-high-risk neuroblastoma.
配对样同源盒2B（）是诊断神经母细胞瘤以及检测神经母细胞瘤微小残留病的一种高度敏感且特异的生物标志物。据我们所知，在新诊断的极低危、低危和中危神经母细胞瘤患者的骨髓（BM）和外周血（PB）样本中表达的临床意义仍不清楚。采用逆转录定量聚合酶链反应（RTqPCR）验证新诊断神经母细胞瘤患者配对的BM和PB样本中的表达水平。在132例患者中，26例BM样本呈现阳性表达（19.7%），11例PB样本呈现阳性表达（8.3%）。在年龄<18个月、国际神经母细胞瘤风险组分期系统为M期和MS期、1p杂合性缺失以及乳酸脱氢酶、血清铁蛋白和神经元特异性烯醇化酶水平较高的患者的BM和PB样本中高表达（<0.05）。在所有符合条件的患者中，2年无事件生存率（EFS）和总生存率（OS）分别为94.7±2.0%和97.7±1.3%。然而，BM和PB样本中表达阳性的患者2年EFS率分别显著降至76.9±8.3%和63.6±14.5%（<0.05）。同样，BM和PB样本中表达阳性的患者2年OS率也分别降至88.5±6.3%和81.8±11.6%（<0.05）。总之，诊断时BM和PB样本中表达阳性对非高危神经母细胞瘤患者具有强烈的不良预后影响。
