Intra-articular extra-axial chordoma of the wrist: a case report with review of the current literature

  • Jan NeumannEmail author
  • Alexandra S. Gersing
  • Thomas F. Barth
  • Melanie Boxberg
  • Klaus Woertler
Case Report


Chordomas are rare bone malignancies that are thought to arise from remnants of the notochord and usually are located in the axial skeleton. Immunophenotypical matching neoplasms primarily found in appendicular locations, referred to as extra-axial chordoma, are rarely encountered by radiologists, surgeons, and pathologists. Only a few of these cases have been described in the literature with only one intra-articular case with involvement of the knee joint. We present the first case of an intra-articular extra-axial chordoma of the wrist. Diagnostic imaging patterns were initially ambiguous and histopathological reprocessing was crucial in order to determine the diagnosis of an intra-articular neoplasm with co-expression of cytokeratins, S-100 protein, and brachyury.


Extra-axial chordoma Intra-articular Wrist Brachyury Notochord 



We would like to thank Dr. H.-P. Engels – RIZ – Die Radiologen, Augsburg, Germany, for providing the radiological images included in this manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.


  1. 1.
    Walcott BP, Nahed BV, Mohyeldin A, Coumans J-V, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012;13:e69–76. Scholar
  2. 2.
    Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO classification of tumours of soft tissue and bone. Lyon: IARC Press; 2013. p. 328–9.Google Scholar
  3. 3.
    Tirabosco R, Mangham DC, Rosenberg AE, et al. Brachyury expression in extra-axial skeletal and soft tissue chordomas: a marker that distinguishes chordoma from mixed tumor/myoepithelioma/parachordoma in soft tissue. Am J Surg Pathol. 2008;32:572–80. Scholar
  4. 4.
    Laskowski J. Pathology of tumors. In: Kolodziejska H, editor. Zarys onkologii. Warsaw: PZWL; 1955. p. 91–9.Google Scholar
  5. 5.
    Kurzawa P, Fundowicz M, Dopierała M, Larque AB, Nielsen GP. Primary extra-axial, para-articular chordoma of the knee. A case report and the review of literature. Histopathology. 2018;72:883–5. Scholar
  6. 6.
    Righi A, Sbaraglia M, Gambarotti M, et al. Extra-axial chordoma: a clinicopathologic analysis of six cases. Virchows Arch. 2018;472:1015–20. Scholar
  7. 7.
    Bitzer A, McCarthy EF, Morris CD. Extra-axial chordoma of the hand. J Hand Surg. 2017;42:933.e1–5. Scholar
  8. 8.
    Tsukamoto S, Vanel D, Righi A, Donati DM, Errani C. Parosteal extra-axial chordoma of the second metacarpal bone: a case report with literature review. Skelet Radiol. 2018;47:579–85. Scholar
  9. 9.
    Huang J, Bhojwani N, Oakley FD, Jordanov MI. Proximal tibial extra-axial chordoma masquerading as renal cell carcinoma metastasis. Skelet Radiol. 2017;46:1567–73. Scholar
  10. 10.
    Rekhi B. Primary, large extra-axial chordoma in proximal tibia: a rare case report with literature review and diagnostic implications. APMIS. 2016;124:238–42. Scholar
  11. 11.
    Lantos JE, Agaram NP, Healey JH, Hwang S. Recurrent skeletal extra-axial chordoma confirmed with brachyury: IMAGING features and review of the literature. Skelet Radiol. 2013;42:1451–9. Scholar
  12. 12.
    Suzuki H, Yamashiro K, Takeda H, Nojima T, Usui M. Extra-axial soft tissue chordoma of wrist. Pathol Res Pract. 2011;207:327–31. Scholar
  13. 13.
    DeComas AM, Penfornis P, Harris MR, Meyer MS, Pochampally RR. Derivation and characterization of an extra-axial chordoma cell line (EACH-1) from a scapular tumor. J Bone Joint Surg Am. 2010;92:1231–40. Scholar
  14. 14.
    O’Donnell P, Tirabosco R, Vujovic S, et al. Diagnosing an extra-axial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation. Skelet Radiol. 2007;36:59–65. Scholar
  15. 15.
    Almefty K, Pravdenkova S, Colli BO, Al-Mefty O, Gokden M. Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 2007;110:2457–67. Scholar
  16. 16.
    Stemple DL. Structure and function of the notochord: an essential organ for chordate development. Development (Cambridge, England). 2005;132:2503–12. Scholar
  17. 17.
    Scolyer RA, Bonar SF, Palmer AA, et al. Parachordoma is not distinguishable from axial chordoma using immunohistochemistry. Pathol Int. 2004;54:364–70. Scholar
  18. 18.
    Salisbury JR, Isaacson PG. Demonstration of cytokeratins and an epithelial membrane antigen in chordomas and human fetal notochord. Am J Surg Pathol. 1985;9:791–7.CrossRefGoogle Scholar
  19. 19.
    Folpe AL, Agoff SN, Willis J, Weiss SW. Parachordoma is immunohistochemically and cytogenetically distinct from axial chordoma and extraskeletal myxoid chondrosarcoma. Am J Surg Pathol. 1999;23:1059–67.CrossRefGoogle Scholar
  20. 20.
    Henderson SR, Guiliano D, Presneau N, et al. A molecular map of mesenchymal tumors. Genome Biol. 2005;6:R76. Scholar
  21. 21.
    Vujovic S, Henderson S, Presneau N, et al. Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol. 2006;209:157–65. Scholar
  22. 22.
    Hallor KH, Staaf J, Jönsson G, et al. Frequent deletion of the CDKN2A locus in chordoma: analysis of chromosomal imbalances using array comparative genomic hybridisation. Br J Cancer. 2008;98:434–42. Scholar
  23. 23.
    von Witzleben A, Goerttler LT, Marienfeld R, et al. Preclinical characterization of novel chordoma cell systems and their targeting by pharmocological inhibitors of the CDK4/6 cell-cycle pathway. Cancer Res. 2015;75:3823–31. Scholar
  24. 24.
    Inoue K, Fry EA. Aberrant expression of p16INK4a in human cancers—a new biomarker? Cancer Rep Rev. 2018;2.
  25. 25.
    Witkiewicz AK, Knudsen KE, Dicker AP, Knudsen ES. The meaning of p16(ink4a) expression in tumors: functional significance, clinical associations and future developments. Cell Cycle (Georgetown, Texas). 2011;10:2497–503. Scholar

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© ISS 2019

Authors and Affiliations

  1. 1.Department of Diagnostic and Interventional RadiologyTechnical University of MunichMunichGermany
  2. 2.Institute of PathologyUniversity Hospital UlmUlmGermany
  3. 3.Department of General Pathology and Pathological AnatomyTechnical University of MunichMunichGermany

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