Olkhova L V, Kushel Yu V, Kadyrov Sh U, Melikyan A G, Gorelyshev S K, Popov V E, Schultz E I, Ryzhova M V, Zheludkova O G
Children's Clinical Hospital of the Pirogov Russian National Medical University, Moscow, Russia.
Burdenko Neurosurgical Center, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2021;85(2):17-25. doi: 10.17116/neiro20218502117.
The purpose of this study was to assess the influence of resection quality on overall survival and disease-free survival in children with atypical teratoid-rhabdoid tumors (ATRT). The study included children younger than 18 years old for the period from 2008 to 2019. There were 134 interventions in 105 patients with ATRT including 11 redo resections («second-look» surgery) and 18 procedures for tumor recurrence. Age of patients ranged from 2 to 168 months (median 21 months). Patients with supratentorial tumors prevailed (50.5%), infratentorial neoplasms were diagnosed in 45.7% of patients, spinal cord lesion - 3.8% of cases. At the first stage, all patients underwent surgical treatment. Total resection was achieved in 34 (32.4%) patients, subtotal - 37 (35.2%) patients, partial resection - 30 (28.6%) patients. Biopsy was performed in 4 (3.8%) patients. Quality of resection and age at surgery significantly influenced overall and disease-free survival. Extended resection of tumor followed by adjuvant chemo- and radiotherapy are required to improve survival although ATRTs are high-grade neoplasms with poor prognosis.
本研究的目的是评估手术切除质量对非典型畸胎样/横纹肌样瘤(ATRT)患儿总生存期和无病生存期的影响。该研究纳入了2008年至2019年期间年龄小于18岁的儿童。105例ATRT患者共进行了134次手术干预,其中包括11次再次切除术(“二次探查”手术)和18次肿瘤复发手术。患者年龄范围为2至168个月(中位年龄21个月)。幕上肿瘤患者居多(50.5%),幕下肿瘤患者占45.7%,脊髓病变患者占3.8%。在第一阶段,所有患者均接受了手术治疗。34例(32.4%)患者实现了全切,37例(35.2%)患者为次全切,30例(28.6%)患者为部分切除。4例(3.8%)患者进行了活检。手术切除质量和手术时年龄对总生存期和无病生存期有显著影响。尽管ATRT是预后较差的高级别肿瘤,但仍需要扩大肿瘤切除范围并辅以放化疗以提高生存率。