Gangliogliomas (GGs) are rare, usually low-grade tumors that account for 1–2% of all central nervous system (CNS) neoplasms. Spinal GGs are exceedingly rare (1% of all spinal tumors) and the presentation of anaplastic features in them is even rarer. According to the last World Health Organization (WHO) classification of CNS neoplasms, anaplastic GG (AGG) is classified as a malignant neoplasm (grade III). We performed a scoping review of the literature to elucidate the epidemiology, clinical features, histopathology, treatment, and outcome of primary spinal AGGs, which, to the best of our knowledge, is the first such review. Relevant studies were identified by a search of the MEDLINE and SCOPUS databases, using the following combination of search strings: (anaplastic ganglioglioma or malignant ganglioglioma or high grade ganglioglioma) AND (spine or spinal or spinal cord). We included studies related to primary or recurrent AGGs and malignant transformation of low-grade GGs. The search produced 15 eligible studies, plus two studies from the references, all of which were case reports of patients with spinal AGGs (17 studies with 22 patients). The mean age of the patients was 21.4 years and the sex ratio was 1:1, with male predominance. Motor impairment was the most common presentation, followed by sensory impairment, gait problems, urinary disturbances, and back pain. The thoracic spine was the most frequently involved area (14/22) followed by the cervical (6/22) and lumbar (5/22) spine. In terms of histology, the anaplastic features were usually predominant in the glial element, resembling high-grade astrocytomas, while the neuronal element was composed of the so-called dysplastic ganglion (neuronal) synaptophysin-positive cells, without mitotic figures. Complete surgical resection of the tumor without neurological compromise, plus adjuvant chemotherapy and radiotherapy, was the treatment protocol implemented in the two patients with the best outcome. Primary spinal AGG is an exceedingly rare entity, with only 22 cases being retrieved after an extensive literature search. They appear to affect children and young adults and tend to manifest aggressive behavior. Most studies report that only the glial component of AGGs presents high-grade malignant features, with low mitotic activity in the neuronal component. We therefore suggest that, pending novel targeted therapy, AGGs should be treated as high-grade gliomas, with an aggressive treatment protocol consisting of maximal safe resection and adjuvant chemotherapy and radiotherapy.

神经节胶质瘤 (GG) 是罕见的，通常是低级别肿瘤，占所有中枢神经系统 (CNS) 肿瘤的 1-2%。脊柱 GG 极为罕见（占所有脊柱肿瘤的 1%），其中间变性特征的表现更为罕见。根据世界卫生组织 (WHO) 对 CNS 肿瘤的最新分类，间变性 GG (AGG) 被归类为恶性肿瘤（III 级）。我们对文献进行了范围审查，以阐明原发性脊柱 AGG 的流行病学、临床特征、组织病理学、治疗和结果，据我们所知，这是第一次这样的审查。通过使用以下搜索字符串组合搜索 MEDLINE 和 SCOPUS 数据库，确定了相关研究：（间变性神经节胶质瘤或恶性神经节胶质瘤或高级神经节胶质瘤）和（脊柱或脊髓或脊髓）。我们纳入了与原发性或复发性 AGG 和低级别 GG 恶变相关的研究。搜索产生了 15 项符合条件的研究，以及来自参考文献的两项研究，所有这些都是脊柱 AGG 患者的病例报告（17 项研究，22 名患者）。患者平均年龄为21.4岁，性别比为1:1，以男性为主。运动障碍是最常见的表现，其次是感觉障碍、步态问题、排尿障碍和背痛。胸椎是最常受累的区域（14/22），其次是颈椎（6/22）和腰椎（5/22）。在组织学方面，间变性特征通常在胶质细胞中占主导地位，类似于高级星形细胞瘤，而神经元由所谓的发育不良的神经节（神经元）突触素阳性细胞组成，没有有丝分裂图。在没有神经系统损害的情况下完全手术切除肿瘤，加上辅助化疗和放疗，是在两名患者中实施的治疗方案，结果最好。原发性脊柱 AGG 是一种极为罕见的实体，经过广泛的文献检索后仅检索到 22 例。它们似乎会影响儿童和年轻人，并倾向于表现出攻击性行为。大多数研究报告说，只有 AGG 的神经胶质成分呈现出高度恶性特征，神经元成分中的有丝分裂活性较低。因此，我们建议，在等待新的靶向治疗之前，应将 AGG 视为高级别胶质瘤，