Liu Matthew A, Gendreau Julian L, Loya Joshua J, Brown Nolan J, Keith Amber, Sahyouni Ronald, Abraham Mickey E, Gonda David, Levy Michael L
Department of Neurosurgery, University of California, San Diego, La Jolla, California.
Department of Biomedical Engineering, Johns Hopkins Whiting School of Engineering, Baltimore, Maryland.
J Neurosurg Case Lessons. 2021 Nov 22;2(21):CASE21434. doi: 10.3171/CASE21434.
Chordomas are rare malignant neoplasms that develop from the primitive notochord with < 5% of the tumors occurring in pediatric patients younger than the age of 20. Of these pediatric chordomas, those affecting the craniocervical junction (C1-C2) are even more rare; therefore, parameters for surgical management of these pediatric tumors are not well characterized.
In this case, a 3-year-old male was found to have a clival chordoma on imaging with extension to the craniocervical junction resulting in spinal cord compression. Endoscopic-assisted transoral transclival approach for clival tumor resection was performed first. As a second stage, the patient underwent a left-sided far lateral craniotomy and cervical laminectomy for resection of the skull base chordoma and instrumented fusion of the occiput to C3. He made excellent improvements in strength and dexterity during rehab and was discharged after 3 weeks.
In pediatric patients with chordoma with extension to the craniocervical junction and spinal cord compression, decompression with additional occipito-cervical fusion appears to offer a good clinical outcome. Fusion performed as a separate surgery before or at the same time as the initial tumor resection surgery may lead to better outcomes.
脊索瘤是一种罕见的恶性肿瘤，起源于原始脊索，20岁以下的儿科患者中肿瘤发生率低于5%。在这些儿科脊索瘤中，影响颅颈交界区（C1 - C2）的更为罕见；因此，这些儿科肿瘤的手术治疗参数尚未得到充分描述。
在本病例中，一名3岁男性经影像学检查发现患有斜坡脊索瘤，并延伸至颅颈交界区，导致脊髓受压。首先采用内镜辅助经口经斜坡入路切除斜坡肿瘤。作为第二阶段，患者接受了左侧远外侧开颅手术和颈椎椎板切除术，以切除颅底脊索瘤，并进行枕骨至C3的器械融合。他在康复过程中力量和灵活性有显著改善，3周后出院。
对于患有延伸至颅颈交界区并伴有脊髓受压的儿科脊索瘤患者，额外进行枕颈融合减压似乎能带来良好的临床效果。在初始肿瘤切除手术之前或同时作为单独手术进行融合可能会带来更好的结果。
