van Heerden Jaques, Kruger Mariana, Esterhuizen Tonya, Hendricks Marc, Geel Jennifer, Büchner Ané, Naidu Gita, du Plessis Jan, Vanemmenes Barry, Uys Ronelle, Hadley G P
Paediatric Haematology and Oncology, Department of Paediatrics and Child Health, Faculty of Medicine and Health Sciences, Tygerberg Hospital, Stellenbosch University, Cape Town, South Africa.
Biostatistics Unit, Faculty of Medicine and Health Sciences, Stellenbosch University, Stellenbosch, South Africa.
Pediatr Surg Int. 2020 Apr;36(4):457-469. doi: 10.1007/s00383-020-04627-x. Epub 2020 Feb 28.
To investigate the impact of local therapies on high-risk neuroblastoma (HR-NB) outcomes in South Africa.
Data from 295 patients with HR-NB from nine pediatric oncology units between 2000 and 2014 were analysed. All patients received chemotherapy. Five-year overall (OS) and event free survival (EFS) were determined for patients who had received local therapy, either surgery or radiotherapy or both.
Surgery was performed in only 35.9% (n = 106/295) patients. Surgical excision was done for 34.8% (n = 85/244) of abdominal primaries, 50.0% (n = 11/22) of thoracic primaries; 22.2% (n = 2/9) neck primaries and 66.7% (n = 8/12) of the paraspinal primaries. Only 15.9% (n = 47/295) of all patients received radiotherapy. Children, who had surgery, had an improved five-year OS of 32.1% versus 5.9% without surgery (p < 0.001). Completely resected disease had a five-year OS of 30.5%, incomplete resections 31.4% versus no surgery 6.0% (p < 0.001). Radiated patients had a five-year OS of 21.3% versus 14.2% without radiotherapy (p < 0.001). Patients who received radiotherapy without surgical interventions, had a marginally better five-year OS of 12.5% as opposed to 5.4% (p < 0.001). Patients who underwent surgery had a longer mean overall survival of 60.9 months, while patients, who were irradiated, had a longer mean overall survival of 7.9 months (p < 0.001). On multivariate analysis, complete metastatic remission (p < 0.001), surgical status (p = 0.027), and radiotherapy status (p = 0.040) were significant predictive factors in abdominal primaries.
Surgery and radiotherapy significantly improve outcomes regardless of the primary tumor site, emphasizing the importance of local control in neuroblastoma.
探讨局部治疗对南非高危神经母细胞瘤（HR-NB）治疗结果的影响。
分析了2000年至2014年间来自9个儿科肿瘤治疗单位的295例HR-NB患者的数据。所有患者均接受了化疗。对接受过局部治疗（手术、放疗或两者皆有）的患者确定其5年总生存率（OS）和无事件生存率（EFS）。
仅35.9%（n = 106/295）的患者接受了手术。腹部原发肿瘤的手术切除率为34.8%（n = 85/244），胸部原发肿瘤为50.0%（n = 11/22）；颈部原发肿瘤为22.2%（n = 2/9），脊柱旁原发肿瘤为66.7%（n = 8/12）。所有患者中仅15.9%（n = 47/295）接受了放疗。接受手术的儿童5年总生存率为32.1%，未接受手术的为5.9%（p < 0.001）。完全切除肿瘤的患者5年总生存率为30.5%，不完全切除的为31.4%，未手术的为6.0%（p < 0.001）。接受放疗的患者5年总生存率为21.3%，未接受放疗的为14.2%（p < 0.001）。未接受手术干预仅接受放疗的患者5年总生存率略高，为12.5%，而未接受放疗的为5.4%（p < 0.001）。接受手术的患者平均总生存期更长，为60.9个月，而接受放疗的患者平均总生存期为7.9个月（p < 0.001）。多因素分析显示，完全转移性缓解（p < 0.001）、手术状态（p = 0.027）和放疗状态（p = 0.040）是腹部原发肿瘤的重要预测因素。
无论原发肿瘤部位如何，手术和放疗均能显著改善治疗结果，强调了神经母细胞瘤局部控制的重要性。
