Eco Lourdes C, Brayton Alison, Whitehead William E, Jea Andrew
1Section of Pediatric Neurosurgery, Riley Hospital for Children, Department of Neurological Surgery, Indiana University School of Medicine, Goodman Campbell Brain and Spine, Indianapolis, Indiana; and.
2Division of Pediatric Neurosurgery, Department of Neurosurgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
J Neurosurg Pediatr. 2019 Jul 1;24(1):62-65. doi: 10.3171/2019.2.PEDS18752. Epub 2019 Apr 12.
Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient's treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.
脊索瘤是组织学上为良性但具有局部侵袭性的肿瘤。它们起源于斜坡、活动脊柱和骶骨处脊索的胚胎残余物。脊索瘤在儿童年龄组中是罕见肿瘤。鉴于其倾向于位于难以接近的解剖区域且靠近关键神经血管结构，其手术治疗具有挑战性。虽然整块切除并带有手术切缘一直被倡导为脊索瘤的首选治疗方法，但肿瘤特征以及对相邻解剖边界的侵犯可能不允许对肿瘤进行安全的整块切除。在此，作者报告了一名5岁男孩患有C1脊索瘤且伴有硬膜外和椎体前扩展的病例。患者的治疗包括采用远外侧入路切除肿瘤和C1椎弓，随后使用一个从颅底延伸至C2的可扩张椎间融合器对颅颈交界区进行环形重建，并进行枕颈后路脊柱内固定。术后42个月，患者神经功能保持完好，肿瘤状态稳定，且没有颅颈交界区内固定失败的迹象。
