Hamilton Kimberly, Rebsamen Susan, Salamat Shahriar, Ahmed Raheel
Departments of1Neurological Surgery.
2Radiology, and.
J Neurosurg Pediatr. 2019 Feb 22;23(5):628-633. doi: 10.3171/2018.12.PEDS18544. Print 2019 May 1.
An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Neoplastic transformation of the notochord is reviewed to illustrate the developmental basis for the surgical anatomy and pathogenesis of the classic chordoma variant. Clinical and pathological features are reviewed to differentiate this chordoma presentation from classic osseous chordomas and ecchordosis physaliphora, a related benign developmental notochordal lesion. Finally, the role of developmental signaling in the pathogenesis of chordomas from postembryonic notochordal tissue is discussed.
小儿年龄组骶骨脊索瘤的硬膜内骨外表现迄今尚未见报道。本文报告一例11岁男孩，患有硬膜内骨外骶骨脊索瘤。他接受了全切除手术，并接受了辅助质子束治疗。本文回顾了脊索的肿瘤性转化，以阐明经典脊索瘤变体的手术解剖学和发病机制的发育基础。回顾临床和病理特征，以将这种脊索瘤表现与经典骨脊索瘤和泡状脊索瘤（一种相关的良性发育性脊索病变）区分开来。最后，讨论了发育信号在胚胎后脊索组织来源的脊索瘤发病机制中的作用。
