Department of Pathology and Laboratory Medicine, University of Saskatchewan, Royal University Hospital, 103, Hospital Drive, Saskatoon, S7N 0W8, Canada.
Spine J. 2013 Nov;13(11):e35-43. doi: 10.1016/j.spinee.2013.06.058. Epub 2013 Sep 8.
Chordomas are rare tumors in the craniospinal axis arising from persistent notochordal rests commonly seen in the skull base, including the clivus and the sacrum. Chordomas in the mobile spine occur less commonly. To the best of our knowledge, the clinical presentation of acute cauda equina syndrome (CES) due to chordoma of the lumbar vertebra is not published in the English literature.
To describe an unusual cause of acute CES resulting from chordoma of the lumbar vertebra and discuss management dilemmas in this clinical context.
Case report with review and discussion.
We report the case of a 75-year-old man who presented with acute CES that was clinically considered a metastasis from his previously documented carcinoma of the urinary bladder treated a year ago. Clinical, radiological, and histopathological features of the case and a review of chordomas in the lumbar vertebrae in adults in the published English literature are presented.
He underwent urgent surgical decompression with laminectomy of L3/L4 and L4/L5 with debulking and open biopsy of the tissue mass. Histopathological examination of the tissue mass confirmed the unsuspected diagnosis of chordoma. The salient features of chordomas in the lumbar vertebrae published in the English literature over the last 22 years are summarized. The origin, classification, clinical presentation, and management protocols for lumbar chordomas are also reviewed.
The clinical presentation of acute CES as the first symptom of chordoma in the lumbar vertebrae is extremely rare. Preoperative tissue diagnosis of this uncommon pathology is usually unavailable. In the face of acute CES, surgical decompression remains the primary goal of management with a planned definitive second-stage curative surgical resection for chordoma.
脊索瘤是一种罕见的颅脊柱轴肿瘤，起源于常见于颅底的持续性脊索残余，包括斜坡和骶骨。发生在活动脊柱的脊索瘤则较少见。据我们所知，脊索瘤引起的急性马尾综合征（CES）在英文文献中尚未有报道。
描述一例因腰椎脊索瘤引起的急性 CES 的不常见病因，并讨论该临床情况下的管理难题。
病例报告并结合文献复习和讨论。
我们报告了一例 75 岁男性，因急性 CES 就诊，该 CES 临床考虑为一年前确诊的膀胱癌转移所致。本文介绍了该病例的临床、影像学和组织病理学特征，并对已发表的英文文献中成人腰椎脊索瘤进行了回顾。
患者接受了紧急手术减压，行 L3/L4 和 L4/L5 椎板切除术，并对组织肿块进行了肿瘤切除术和开放性活检。组织肿块的组织病理学检查证实了意外的脊索瘤诊断。对过去 22 年英文文献中发表的腰椎脊索瘤的总结，概括了脊索瘤在腰椎的起源、分类、临床表现和管理方案。
腰椎脊索瘤以急性 CES 为首发症状的临床表现极为罕见。这种罕见病理的术前组织诊断通常不可用。在急性 CES 情况下，手术减压仍然是管理的主要目标，计划进行确定性的第二阶段根治性手术切除脊索瘤。
