Acute cauda equina syndrome secondary to lumbar chordoma: case report and literature review

Abstract

Background context: 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.

Purpose: To describe an unusual cause of acute CES resulting from chordoma of the lumbar vertebra and discuss management dilemmas in this clinical context.

Study design: Case report with review and discussion.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Acute cauda equina syndrome; Benign notochordal cell tumors; Lumbar chordoma; Malignant chordomas; Management dilemmas.