Elsevier

The Spine Journal

Volume 11, Issue 10, October 2011, Pages e7-e10
The Spine Journal

Case Report
Multiple epidural lumbar chordomas without bone involvement in a 17-year-old female: a case report

https://doi.org/10.1016/j.spinee.2011.09.001Get rights and content

Abstract

Background context

Chordomas are rare slow-growing neoplasms or malignant tumors that arise from embryonic rudiments of the primitive notochord. They account for 1% to 4% of all primary malignant bone tumors. Surgery in addition to radiotherapy is often used as the optimum treatment but with marginal effectiveness as these tumors are relatively radioresistant. To the best of our knowledge, there have been no reported cases of multiple and distinct epidural lesions without bone involvement in the literature.

Purpose

To describe an unusual case of two separate epidural chordomas occurring in the lumbar spine without bone involvement.

Study design

Case report.

Methods

Clinical, radiologic, and histopathologic evaluation of a 17-year-old female with two separate adjacent lumbar epidural chordomas without bone involvement treated with complete surgical excision is discussed.

Results

Four years after surgical resection of the tumors, the patient remains asymptomatic, and radiologic results revealed no recurrent lumbar epidural tumor.

Conclusions

Epidural chordomas appear to have a better prognosis due, at least in part, to the ability to more easily completely excise them.

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)

    FDA device/drug status: Not applicable.

    Author disclosures: SLS: Nothing to disclose. IAI: Nothing to disclose. CLM: Nothing to disclose. PJA: Nothing to disclose.

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