Human Pathology
Volume 41, Issue 5 , Pages 653-662 , May 2010

Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and β-catenin, Sox9, and osteocalcin immunostaining of 22 cases

  • Julie C. Fanburg-Smith, MD

      Affiliations

    • Department of Orthopedic and Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
    • Corresponding Author InformationCorresponding author. AIPL, Soft Tissue and Orthopedic Pathology, Silver Spring, MD 20910, USA.
    web address
  • ,
  • Aaron Auerbach, MD, MPH

      Affiliations

    • Department of Hematopathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
    • Department of Immunohistochemistry, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
  • ,
  • Jayson S. Marwaha

      Affiliations

    • Department of Orthopedic and Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
  • ,
  • Zengfeng Wang, PhD

      Affiliations

    • Department of Immunohistochemistry, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
  • ,
  • Elisabeth J. Rushing, MD

      Affiliations

    • Department of Neuropathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA

Received 30 July 2009 ,Revised 31 October 2009 ,Accepted 4 November 2009.

References 

  1. Dantonello TM, Int-Veen C, Leuschner I, et al. Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults: experiences of the CWS and COSS study groups. Cancer. 2008;112:2424–2431
  2. Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer. 1986;57:2444–2453
  3. De CR, Migliaccio I, Falleti J, Del Basso De CM, Pettinato G. Congenital intracranial mesenchymal chondrosarcoma: case report and review of the literature in pediatric patients. Pediatr Dev Pathol. 2008;11:309–313
  4. Salvador AH, Beabout JW, Dahlin DC. Mesenchymal chondrosarcoma—observations on 30 new cases. Cancer. 1971;28:605–615
  5. Guccion JG, Font RL, Enzinger FM, Zimmerman LE. Extraskeletal mesenchymal chondrosarcoma. Arch Pathol. 1973;95:336–340
  6. Lockhart R, Menard P, Martin JP, Auriol M, Vaillant JM, Bertrand JC. Mesenchymal chondrosarcoma of the jaws. Report of four cases. Int J Oral Maxillofac Surg. 1998;27:358–362
  7. Vencio EF, Reeve CM, Unni KK, Nascimento AG. Mesenchymal chondrosarcoma of the jaw bones: clinicopathologic study of 19 cases. Cancer. 1998;82:2350–2355
  8. Rushing EJ, Armonda RA, Ansari Q, Mena H. Mesenchymal chondrosarcoma: a clinicopathologic and flow cytometric study of 13 cases presenting in the central nervous system. Cancer. 1996;77:1884–1891
  9. Salvati M, Caroli E, Frati A, et al. Central nervous system mesenchymal chondrosarcoma. J Exp Clin Cancer Res. 2005;24:317–324
  10. Scheithauer BW, Rubinstein LJ. Meningeal mesenchymal chondrosarcoma: report of 8 cases with review of the literature. Cancer. 1978;42:2744–2752
  11. Fletcher CD, Krausz T. Cartilaginous tumours of soft tissue. Appl Pathol. 1988;6:208–220
  12. Lefebvre V. Toward understanding SOX9 function in chondrocyte differentiation. Matrix Biol. 1998;16:529–540
  13. Soderstrom M, Bohling T, Ekfors T, Nelimarkka L, Aro HT, Vuorio E. Molecular profiling of human chondrosarcomas for matrix production and cancer markers. Int J Cancer. 2002;100:144–151
  14. Wehrli BM, Huang W, de CB, Ayala AG, Czerniak B. Sox9, a master regulator of chondrogenesis, distinguishes mesenchymal chondrosarcoma from other small blue round cell tumors. Hum Pathol. 2003;34:263–269
  15. Akiyama H, Lyons JP, Mori-Akiyama Y, et al. Interactions between Sox9 and beta-catenin control chondrocyte differentiation. Genes Dev. 2004;18:1072–1087
  16. Yano F, Kugimiya F, Ohba S, et al. The canonical Wnt signaling pathway promotes chondrocyte differentiation in a Sox9-dependent manner. Biochem Biophys Res Commun. 2005;333:1300–1308
  17. Ng TL, Gown AM, Barry TS, et al. Nuclear beta-catenin in mesenchymal tumors. Mod Pathol. 2005;18:68–74
  18. Fanburg JC, Rosenberg AE, Weaver DL, et al. Osteocalcin and osteonectin immunoreactivity in the diagnosis of osteosarcoma. Am J Clin Pathol. 1997;108:464–473
  19. Fanburg-Smith JC, Bratthauer GL, Miettinen M. Osteocalcin and osteonectin immunoreactivity in extraskeletal osteosarcoma: a study of 28 cases. Hum Pathol. 1999;30:32–38
  20. Hsu SM, Raine L, Fanger H. Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabeled antibody (PAP) procedures. J Histochem Cytochem. 1981;29:577–580
  21. de Crombrugghe B, Lefebvre V, Nakashima K. Regulatory mechanisms in the pathways of cartilage and bone formation. Curr Opin Cell Biol. 2001;13:721–727
  22. 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
  23. Dabska M, Huvos AG. Mesenchymal chondrosarcoma in the young. Virchows Arch A Pathol Anat Histopathol. 1983;399:89–104
  24. Aigner T, Loos S, Muller S, Sandell LJ, Unni KK, Kirchner T. Cell differentiation and matrix gene expression in mesenchymal chondrosarcomas. Am J Pathol. 2000;156:1327–1335
  25. Ushigome S, Takakuwa T, Shinagawa T, Takagi M, Kishimoto H, Mori N. Ultrastructure of cartilaginous tumors and S-100 protein in the tumors. With reference to the histogenesis of chondroblastoma, chondromyxoid fibroma and mesenchymal chondrosarcoma. Acta Pathol Jpn. 1984;34:1285–1300
  26. Hoang MP, Suarez PA, Donner LR, et al. Mesenchymal chondrosarcoma: a small cell neoplasm with polyphenotypic differentiation. Int J Surg Pathol. 2000;8:291–301
  27. Swanson PE, Lillemoe TJ, Manivel JC, Wick MR. Mesenchymal chondrosarcoma. An immunohistochemical study. Arch Pathol Lab Med. 1990;114:943–948
  28. Lefebvre V, Huang W, Harley VR, Goodfellow PN, de CB. SOX9 is a potent activator of the chondrocyte-specific enhancer of the pro alpha1(II) collagen gene. Mol Cell Biol. 1997;17:2336–2346
  29. Kulyk WM, Franklin JL, Hoffman LM. Sox9 expression during chondrogenesis in micromass cultures of embryonic limb mesenchyme. Exp Cell Res. 2000;255:327–332
  30. Bi W, Deng JM, Zhang Z, Behringer RR, de CB. Sox9 is required for cartilage formation. Nat Genet. 1999;22:85–89
  31. Mau E, Whetstone H, Yu C, Hopyan S, Wunder JS, Alman BA. PTHrP regulates growth plate chondrocyte differentiation and proliferation in a Gli3 dependent manner utilizing hedgehog ligand dependent and independent mechanisms. Dev Biol. 2007;305:28–39
  32. Han Y, Lefebvre V. L-Sox5 and Sox6 drive expression of the aggrecan gene in cartilage by securing binding of Sox9 to a far-upstream enhancer. Mol Cell Biol. 2008;28:4999–5013
  33. Issack PS, Helfet DL, Lane JM. Role of Wnt signaling in bone remodeling and repair. HSSJ. 2008;4:66–70
  34. Topol L, Chen W, Song H, Day TF, Yang Y. Sox9 inhibits Wnt signaling by promoting beta-catenin phosphorylation in the nucleus. J Biol. 2009;284:3323–3333
  35. Hill TP, Spater D, Taketo MM, Birchmeier W, Hartmann C. Canonical Wnt/betacatenin signaling prevents osteoblasts from differentiating into chondrocytes. Dev Cel. 2005;8:727–738

PII: S0046-8177(09)00409-2

doi: 10.1016/j.humpath.2009.11.006

Human Pathology
Volume 41, Issue 5 , Pages 653-662 , May 2010