Tsuchiya Takahiro, Ohno Makoto, Watanabe Yuko, Fujita Shohei, Miyazaki Bunpei, Sugino Hirokazu, Igaki Hiroshi, Yoshida Akihiko, Takahashi Masamichi, Yanagisawa Shunsuke, Osawa Sho, Ogawa Chitose, Narita Yoshitaka
Departments of Neurosurgery and Neuro-Oncology, National Cancer Center Hospital, Tokyo, Japan.
Departments of Pediatric Oncology, National Cancer Center Hospital, Tokyo, Japan.
J Neurosurg Case Lessons. 2024 Aug 12;8(7). doi: 10.3171/CASE24219.
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.
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.
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.
非典型畸胎样/横纹肌样瘤（AT/RT）是一种罕见的恶性肿瘤，很少发生于脊柱间隙，尤其是马尾神经。仅报道过8例小儿马尾神经AT/RT病例。因此，其临床行为及最佳治疗方案仍不明确。
作者描述了一名9岁男孩的病例，该男孩出现进行性背痛和左腿疼痛。最初的磁共振成像显示L3 - 4水平硬膜内髓外病变，1个月内迅速进展至L2 - 5水平。他接受了L2 - 5椎板切除术及肿瘤部分切除术。组织病理学诊断为AT/RT。他接受了辅助化疗和放疗，术后步态障碍有所改善。随访6个月时，未观察到疾病复发。
尽管极为罕见，但AT/RT应纳入鉴别诊断以进行及时的治疗干预。在功能损害最小的情况下进行安全切除，随后进行术后放化疗，可实现肿瘤控制并改善神经功能。https://thejns.org/doi/10.3171/CASE24219
