Choi Ho Yong, Kim Kyung Hyun, Cho Byung-Kyu, Wang Kyu-Chang, Phi Ji Hoon, Lee Ji Yeoun, Park Sung-Hye, Kim Seung-Ki
Department of Neurosurgery, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea.
Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
J Korean Neurosurg Soc. 2021 Jul;64(4):592-607. doi: 10.3340/jkns.2020.0243. Epub 2021 Apr 16.
Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs.
Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately.
The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447).
Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.
关于小儿原发性脊髓肿瘤（PSCT）的研究较少。本研究的目的是对PSCT进行描述性分析和详细的生存分析。
1985年至2017年期间，126例患有PSCT的小儿患者（男∶女，56∶70）在单一机构接受了手术。我们回顾性分析了有关人口统计学、肿瘤特征、结局和生存统计数据。分别对髓内（IM）肿瘤和硬膜外（ED）肿瘤进行亚组分析。
参与者的平均年龄为6.4±5.04岁，平均随访时间为69.5±46.30个月。最常见的部位是ED部位（n = 57，45.2%），其次是IM（n = 43，34.1%）和硬脊膜内髓外（IDEM；n = 16，12.7%）部位。大约一半的PSCT是恶性的（n = 69，54.8%）。最常见的病理类型是神经鞘瘤（n = 14）和神经母细胞瘤（n = 14）。22例患者（17.5%）死于该疾病，平均疾病持续时间为15.8±15.85个月。36例患者（28.6%）病情进展，平均时间为22.6±30.81个月。10年总生存率（OS）和无进展生存率（PFS）分别为81%和66%。对于IM肿瘤，10年OS率和PFS率分别为79%和57%。在ED肿瘤中，10年OS率和PFS率分别为80%和81%。病理和切除范围对总PSCT、IM肿瘤和ED肿瘤的OS均显示出有益影响。PFS在总PSCT和ED肿瘤中受切除范围和病理的影响；然而，在IM肿瘤中，病理是PFS的主要决定因素而非切除范围。改良的麦考密克量表的改善程度显示，实现全切的IM肿瘤患者有改善趋势（p = 0.447）。
大约一半的PSCT是恶性的，ED肿瘤最常见。最常见的病理类型是神经鞘瘤和神经母细胞瘤。病理和切除范围对OS均有决定性影响。对于IM肿瘤，病理是PFS的主要决定因素而非切除范围。IM肿瘤的根治性切除可能是一种可行的选择，可提高生存率且不会增加功能结局恶化的风险。
