PDF(Pubmed)
Abstract：
BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is an uncommon malignant neoplasm and rarely occurs in the spinal space, especially in the cauda equina. Only 8 cases of pediatric AT/RT of the cauda equina have been reported. Therefore, its clinical behavior and optimal treatment remain unclear.
METHODS: The authors describe the case of a 9-year-old boy who presented with progressive back and left leg pain. Initial magnetic resonance imaging showed an intradural extramedullary lesion at the L3-4 level, which progressed rapidly to the L2-5 level within a month. He underwent partial resection of the tumor with an L2-5 laminectomy. The histopathological diagnosis was AT/RT. He received adjuvant chemotherapy and radiotherapy, and his gait disturbance improved postoperatively. At 6 months\' follow-up, disease recurrence was not observed.
CONCLUSIONS: Although extremely rare, AT/RT should be included in the differential diagnosis for prompt therapeutic intervention. Safe resection with minimal functional impairment, followed by postoperative chemoradiation, can lead to tumor control and improve neurological function. https://thejns.org/doi/10.3171/CASE24219.
METHODS: The authors describe the case of a 9-year-old boy who presented with progressive back and left leg pain. Initial magnetic resonance imaging showed an intradural extramedullary lesion at the L3-4 level, which progressed rapidly to the L2-5 level within a month. He underwent partial resection of the tumor with an L2-5 laminectomy. The histopathological diagnosis was AT/RT. He received adjuvant chemotherapy and radiotherapy, and his gait disturbance improved postoperatively. At 6 months\' follow-up, disease recurrence was not observed.
CONCLUSIONS: Although extremely rare, AT/RT should be included in the differential diagnosis for prompt therapeutic intervention. Safe resection with minimal functional impairment, followed by postoperative chemoradiation, can lead to tumor control and improve neurological function. https://thejns.org/doi/10.3171/CASE24219.
摘要：
背景：非典型畸胎样/横纹肌样瘤（AT/RT）是一种罕见的恶性肿瘤，很少发生在脊柱间隙，尤其是在马尾.仅报道了8例小儿马尾草AT/RT。因此,其临床行为和最佳治疗仍不清楚。
方法：作者描述了一个9岁男孩出现进行性背部和左腿疼痛的案例。最初的磁共振成像显示L3-4级别的硬膜内髓外病变，在一个月内迅速发展到L2-5水平。他接受了L2-5椎板切除术的部分切除肿瘤。病理诊断为AT/RT。他接受了辅助化疗和放疗，他的步态障碍在术后有所改善。在6个月的随访中，未观察到疾病复发。
结论：虽然极为罕见，鉴别诊断中应包括AT/RT，以便及时进行治疗干预。具有最小功能损害的安全切除，术后放化疗,可以导致肿瘤控制和改善神经功能。https://thejns.org/doi/10.3171/CASE24219。
方法：作者描述了一个9岁男孩出现进行性背部和左腿疼痛的案例。最初的磁共振成像显示L3-4级别的硬膜内髓外病变，在一个月内迅速发展到L2-5水平。他接受了L2-5椎板切除术的部分切除肿瘤。病理诊断为AT/RT。他接受了辅助化疗和放疗，他的步态障碍在术后有所改善。在6个月的随访中，未观察到疾病复发。
结论：虽然极为罕见，鉴别诊断中应包括AT/RT，以便及时进行治疗干预。具有最小功能损害的安全切除，术后放化疗,可以导致肿瘤控制和改善神经功能。https://thejns.org/doi/10.3171/CASE24219。
