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Review
. 2019 Sep 4;20(1):408.
doi: 10.1186/s12891-019-2799-2.

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

Chih-Wei Chen  1   2 , I-Hsin Chen  1 , Ming-Hsiao Hu  1 , Jen-Chieh Lee  3 , Hsuan-Ying Huang  4 , Ruey-Long Hong  5 , Shu-Hua Yang  6
Affiliations
Review

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

Chih-Wei Chen et al. BMC Musculoskelet Disord. .

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.

Keywords: Extramedullary; Intradural; Mesenchymal chondrosarcoma; Spine.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Preoperative MR images. a Sagittal T1-weighted image (T1WI), b Sagittal T2-weighted image (T2WI), c Sagittal T1WI with gadolinium enhancement, and d Axial T1WI with gadolinium enhancement. An intradural extrameduallary mass located at T3 level (arrows) and was characterized by intermediate signal intensity at T1WI, mildly increased signal intensity at T2WI and evident enhancement after gadolinium administration. Severe spinal cord compression by the tumor was shown (d)
Fig. 2
Fig. 2
a At the × 100 magnification, the tumor is hypercellular and composed of spindle and round cells with a high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. b At the × 200 magnification, the tumor cells have ovoid to round nuclei and inconspicuous cytoplasm, arranged in vague fascicles. Note the eosinophilic chondroid matrix (left field). c RT-PCR confirmed the presence of HEY1-NCOA2 fusion
See this image and copyright information in PMC

References

    1. Fletcher DM, Unni KK, Mertens F. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2002.
    1. Derenda M, Borof D, Kowalina I, Wesolowski W, Kloc W, Iżycka-Świeszewska E. Primary spinal intradural mesenchymal chondrosarcoma with several local regrowths treated with osteoplastic laminotomies: a case report. Surg J (NY) 2017;3:e117–e123. doi: 10.1055/s-0037-1604159. - DOI - PMC - PubMed
    1. Wang L, Motoi T, Khanin R, Olshen A, Mertens F, Bridge J, et al. Identification of a novel recurrent HEY1-NCOA2 fusion in mesenchymal chondrosarcoma based on a genome-wide screen of exon-level expression data. Genes Chromosom Cancer. 2012;51:127–139. doi: 10.1002/gcc.20937. - DOI - PMC - PubMed
    1. Ogden AT, McCormick PC. Intradural extramedullary spinal tumors. In: Bridwell KH, DeWald RL, editors. The textbook of spinal surgery. 3. Philadelphia: Lippincott Williams & Wilkins; 2011. pp. 1535–1543.
    1. Lightenstein L, Bernstein D. Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer. 1959;12:1142–1157. doi: 10.1002/1097-0142(195911/12)12:6<1142::AID-CNCR2820120610>3.0.CO;2-D. - DOI - PubMed

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