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01.12.2019 | Case report | Ausgabe 1/2019 Open Access

BMC Musculoskeletal Disorders 1/2019

Primary intradural extramedullary spinal mesenchymal chondrosarcoma: case report and literature review

Zeitschrift:
BMC Musculoskeletal Disorders > Ausgabe 1/2019
Autoren:
Chih-Wei Chen, I-Hsin Chen, Ming-Hsiao Hu, Jen-Chieh Lee, Hsuan-Ying Huang, Ruey-Long Hong, Shu-Hua Yang
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Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1186/​s12891-019-2799-2) contains supplementary material, which is available to authorized users.
Chih-Wei Chen and I-Hsin Chen contributed equally to this work.

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Abstract

Background

Mesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is exceedingly rare and usually found in pediatric patients. Herein, we present an elderly patient with primary intradural extramedullary spinal MCS. Relevant literatures are reviewed to disclose characteristics of intradural extramedullary spinal MCS.

Case presentation

A 64-year-old female presented with urinary difficulty and tightness of upper back preceding progressive weakness of right lower extremity. Magnetic resonance imaging revealed an intradural extramedullary tumor at the level of 3rd thoracic vertebra. This patient underwent total tumor resection and then received adjuvant radiotherapy. Histopathological examination showed that the tumor composed of spindle and round cells with high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. Along with immunohistochemical findings and the existence of HEY1-NCOA2 fusion transcript, the diagnosis of MCS was confirmed. Neurologic deficit recovered nearly completely after surgery. No evidence of local recurrence or distant metastasis was found 5 years after treatments. Including the current case, a total of 18 cases have been reported in the literature with only one case with local recurrence and one case of mortality. The current case was the eldest patient diagnosed with primary intraspinal MCS in the literature.

Conclusions

MCS rarely appears in the intradural space of the spine. In contrast to classic MCS, treatment outcome of primary intradural extramedullary spinal MCS is usually excellent as total tumor resection is commonly achievable. Adjuvant radiotherapy may reduce local recurrence and chemotherapy may be associated with fewer recurrences especially for unresectable tumors.
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