Schneider Christian, Vosbeck Jürg, Grotzer Michael A, Boltshauser Eugen, Kothbauer Karl F
Division of Neurosurgery, Cantonal Hospital of Lucerne, Lucerne, Switzerland.
Pediatr Neurosurg. 2012;48(1):42-7. doi: 10.1159/000339851. Epub 2012 Aug 21.
Gangliogliomas (GGs) are a small subset of intramedullary spinal cord tumors in children. The anaplastic variant (WHO grade III) appears to be an extreme rarity. A literature research revealed only 15 case reports of intramedullary anaplastic GGs (aGGs) and only 4 pediatric patients. The course of an 18-month-old boy with sudden onset of paraparesis is presented. Spinal MRI revealed a contrast-enhancing intramedullary tumor ranging from T6 to T12. The patient underwent a standard laminectomy/laminoplasty and gross total resection of the lesion. His neurological status remained unchanged postoperatively and he recovered very well during outpatient neurorehabilitation. Neuropathologic examination revealed an aGG of WHO grade III. Because of the high-grade histology, adjuvant radiotherapy and chemotherapy with temozolomide were administered. The patient subsequently recovered to a normal functional status. Clinical and radiographic progression-free survival is now 4 years. Based on an extensive literature review, this is only the fifth pediatric patient with a primary intramedullary aGG and the second with documented progression-free survival of over 4 years. Another 4 primary intramedullary aGGs in adults and 7 patients with spinal dissemination from a cerebral aGG or malignant transformation of a low-grade GG have been reported. In comparison to the published case reports, which often indicate significant neurological dysfunction and rather short survival, the neurological recovery in this patient was favorable, and the oncologic outcome even more so. This is an argument for the use of the aggressive treatment regimen of complete resection followed by radio- and chemotherapy applied here.
神经节胶质瘤（GGs）是儿童脊髓髓内肿瘤中的一小部分。间变性变体（世界卫生组织III级）似乎极为罕见。文献研究仅发现15例脊髓髓内间变性GGs（aGGs）的病例报告，其中仅有4例为儿科患者。本文介绍了一名18个月大男孩突然出现双下肢轻瘫的病例。脊髓MRI显示一个从T6到T12的强化髓内肿瘤。患者接受了标准的椎板切除术/椎板成形术，并对病变进行了全切除。术后其神经状态未改变，在门诊神经康复期间恢复良好。神经病理学检查显示为世界卫生组织III级的aGG。由于组织学分级高，给予了辅助放疗和替莫唑胺化疗。患者随后恢复到正常功能状态。目前临床和影像学无进展生存期为4年。基于广泛的文献综述，这是第五例原发性脊髓髓内aGG的儿科患者，也是第二例有记录的无进展生存期超过4年的患者。另外还报告了4例成人原发性脊髓髓内aGG以及7例因脑aGG脊髓播散或低级别GG恶变的患者。与已发表的病例报告相比，那些报告往往显示出明显的神经功能障碍且生存期较短，而该患者的神经功能恢复良好，肿瘤学结局更是如此。这支持了在此采用完整切除后进行放疗和化疗的积极治疗方案。
