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青少年硬膜内脊索瘤:病例报告

Juvenile intradural chordoma: case report.

作者信息

Chang Steven W, Gore Pankaj A, Nakaji Peter, Rekate Harold L

机构信息

Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ 85013, USA.

出版信息

Neurosurgery. 2008 Feb;62(2):E525-6; discussion E527. doi: 10.1227/01.neu.0000316022.74162.00.

DOI:10.1227/01.neu.0000316022.74162.00
PMID:18382292
Abstract

OBJECTIVE

We report the youngest known case of a prepontine intradural chordoma. These tumors are exceedingly rare. Unlike their more common extradural counterparts, no recurrence of an intradural chordoma has been reported.

CLINICAL PRESENTATION

A 9-year-old boy underwent diagnostic imaging for evaluation of headaches. Although neurologically intact, a magnetic resonance imaging scan revealed a large prepontine mass with focal enhancement.

INTERVENTION

Endoscopic-assisted gross total resection was attained with staged bilateral retrosigmoid approaches. There were no additional adjuvant therapies. At the time of the 1-year follow-up evaluation, the patient had no recurrence.

CONCLUSION

By using an endoscopic-assisted procedure, we achieved complete resection of an intradural chordoma offering a potential for surgical cure. Resection is particularly advantageous because it spares the young child the need for radiation treatment. Close follow-up is warranted because we postulate that this tumor exists in a biological continuum between benign notochordal hamartomatous remnants and typical invasive chordomas.

摘要

目的

我们报告了已知最年轻的桥前池硬脊膜内脊索瘤病例。这些肿瘤极为罕见。与更常见的硬膜外脊索瘤不同,尚未有硬膜内脊索瘤复发的报道。

临床表现

一名9岁男孩因头痛接受诊断性影像学检查。尽管神经功能完好,但磁共振成像扫描显示桥前有一个大肿块,有局灶性强化。

干预措施

采用分期双侧乙状窦后入路,在内镜辅助下实现了肿瘤全切除。未进行额外的辅助治疗。在1年的随访评估时,患者未复发。

结论

通过内镜辅助手术,我们实现了硬膜内脊索瘤的完全切除,为手术治愈提供了可能。切除尤其具有优势,因为它使幼儿无需接受放射治疗。鉴于我们推测这种肿瘤存在于良性脊索错构瘤残余与典型侵袭性脊索瘤之间的生物学连续体中,因此有必要进行密切随访。

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