Case ReportMultiple epidural lumbar chordomas without bone involvement in a 17-year-old female: a case report
Introduction
Chordomas are rare slow-growing neoplasms that arise from embryonic rudiments of the primitive notochord [1]. They account for 1% to 4% of all primary malignant bone tumors [2], [3], [4]. Symptoms often present only after the lesion becomes very large, often with extensive local invasion around the adjacent neurovascular structures. This makes complete excision without considerable morbidity difficult in most cases. Yet, because of its unresponsiveness to chemotherapy and traditional radiation, the preferred treatment for chordomas remains total resection.
Chordomas have been reported to arise from a purely epidural location without bony extension [5]. These epidural chordomas appear to have better prognosis due, at least in part, to the ability to more easily completely excise these lesions. We describe an unusual case of two separate epidural chordomas occurring in the lumbar spine that were completely excised in a patient who did not undergo any adjuvant treatment and has not had any evidence of recurrence 4 years postoperatively.
Section snippets
Case report
A 17-year-old female presented with a 2-month history of progressive bilateral leg pain. This shooting pain originates in her buttock bilaterally and radiates into her posterolateral thigh and calves bilaterally. Standing and valsalva exacerbate this pain, whereas lying flat with her legs raised ameliorates this pain. She denied any associated weakness, paresthesias, or numbness. She initially presented to the local emergency room, where a computed tomography (CT) scan was obtained but did not
Discussion
Chordomas can occur at any age but do so primarily in adults. They are most prevalent in patients older than 40 years, with a male-to-female ratio of 1.7:3.1 [1]. Fifty percent of chordomas arise in the sacroccygeal region, 15% in the sphenooccipital region, whereas the remaining 15% are vertebral chordomas and rare examples of extranotochordal chordomas [6], [7], [8], [9]. In children, the distribution is different. Of them, 63% occur in the sphenooccipital region, 21% in the sacroccygeal
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Cited by (4)
A rare case of epidural chordoma without bone involvement within the thoracic spinal canal: A case report with a review of literature
2023, Journal of Orthopaedic ScienceCitation Excerpt :An extraosseous chordoma of the spine with no bone involvement was first reported by Yuh et al., in 1989; it was a lumbar epidural chordoma with typical MRI findings [9]. To the best of our knowledge, there have been 21 cases of extraosseous chordomas of the spine with no bone involvement reported in the English literature to date (see Table 1) [8–25]. Only three of them were extraosseous chordomas located around the thoracic region, with one case each occurring in the intradural, epidural, and paravertebral regions [11,13,25].
Sacral epidural chordoma
2016, Spine JournalChordoma of the posterior mediastinum accompanied by synchronous lesion
2017, Radiologia BrasileiraSpine extra-osseous chordoma mimicking neurogenic tumors: Report of three cases and review of the literatures
2016, World Journal of Surgical Oncology
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Author disclosures: SLS: Nothing to disclose. IAI: Nothing to disclose. CLM: Nothing to disclose. PJA: Nothing to disclose.