Pediatric extraosseous sacral chordoma: case report and literature review of embryonic derivation and clinical implications

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An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Neoplastic transformation of the notochord is reviewed to illustrate the developmental basis for the surgical anatomy and pathogenesis of the classic chordoma variant. Clinical and pathological features are reviewed to differentiate this chordoma presentation from classic osseous chordomas and ecchordosis physaliphora, a related benign developmental notochordal lesion. Finally, the role of developmental signaling in the pathogenesis of chordomas from postembryonic notochordal tissue is discussed.

ABBREVIATIONS EP = ecchordosis physaliphora; GTR = gross-total resection; SHH = sonic hedgehog; TGF = transforming growth factor.

Article Information

Correspondence Raheel Ahmed: University of Wisconsin, Madison, WI.

INCLUDE WHEN CITING Published online February 22, 2019; DOI: 10.3171/2018.12.PEDS18544.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.



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    MRI showing the extraosseous sacral location of the chordoma. Preoperative sagittal T2-weighted (A), precontrast T1-weighted (B), and postcontrast T1-weighted (C) images are shown. The tumor demonstrated mixed signal hyperintensity on T2 (A), mixed iso- to hyperintense signal on precontrast T1 (B), and heterogeneous enhancement of the isointense soft-tissue component after contrast administration (C). Axial T2-weighted (D) and postcontrast T1-weighted (E) images show the lesion extending into the proximal neural foramen, scalloping the dorsal ventral body at L5 and S1. Intermediate diffusion restriction is demonstrated on the apparent diffusion coefficient composite map (F and G). Postoperative sagittal T2-weighted (H), precontrast T1-weighted (I), and postcontrast T1-weighted (J) images at follow-up show no evidence of residual tumor or recurrence.

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    Photomicrographs stained using H & E reveal sheets and chords of epithelioid cells with pink cytoplasm and abundance of extracellular mucoid matrix (A). The tumor cells reveal strong nuclear immunolabeling with brachyury antibody (B). Original magnification ×20. Figure is available in color online only.





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