Spinal neurocytoma (SN), although frequently reportedly as tumors of the central nervous system (CNS), are a distinct class of tumors, which can achieve a better prognosis following subtotal or gross total tumor resection. Nonetheless, even with the premise of successful treatment after tumor resection, poor prognosis after treatment due to the SN high proliferation index (typically known as atypical SN) have been reported. Over the past two decades, atypical SN was only reported in four pediatric cases, amidst the lingering controversy surrounding its postoperative adjuvant therapy. Thus, herein, we report a unique case of atypical SN with epidermal growth factor receptor (EGFR) amplification mutation in a 12-year-old boy. We, however, also highlighted the significance of radiotherapy and target therapy for patients with SN.

脊髓神经细胞瘤 (SN)，虽然经常被报道为中枢神经系统 (CNS) 的肿瘤，但它是一类独特的肿瘤，在肿瘤次全切除或大体全切除后可以获得更好的预后。尽管如此，即使在肿瘤切除后成功治疗的前提下，由于 SN 高增殖指数（通常称为非典型 SN）导致治疗后预后不良也有报道。在过去的 20 年中，非典型 SN 仅在 4 例儿科病例中报告，围绕其术后辅助治疗的争议挥之不去。因此，在此，我们报告了一个 12 岁男孩具有表皮生长因子受体 (EGFR) 扩增突变的非典型 SN 的独特病例。然而，我们也强调了放射治疗和靶向治疗对 SN 患者的重要性。