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Biology of Conventional Chondrosarcoma

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Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions

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Abstract

Conventional chondrosarcoma (CS) is a drug-resistant tumour and the detection of new therapeutic markers is mandatory for advanced/metastatic disease. It is characterized by a complex karyotype associated with a genetic instability that increases by increasing histological grade. Currently the most reliable predictive factors are age and grade, but many agents are under investigation to improve CS patient survival.

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References

  1. Szuhai K, Cleton-Jansen AM, Hogendoorn PC, Bovée JV. Molecular pathology and its diagnostic use in bone tumors. Cancer Genet. 2012;205:193–204.

    Article  CAS  Google Scholar 

  2. Pansuriya TC, van Eijk R, d’Adamo P, van Ruler MA, Kuijjer ML, Oosting J, Cleton-Jansen AM, van Oosterwijk JG, Verbeke SL, Meijer D, et al. Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome. Nat Genet. 2011;43:1256–61.

    Article  CAS  Google Scholar 

  3. Waitkus MS, Diplas BH, Yan H. Biological role and therapeutic potential of IDH mutations in cancer. Cancer Cell. 2018;34(2):186–95. https://doi.org/10.1016/j.ccell.2018.04.011.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Amary MF, Bacsi K, Maggiani F, Damato S, Halai D, Berisha F, Pollock R, O’Donnell P, Grigoriadis A, Diss T, Eskandarpour M, Presneau N, Hogendoorn PC, Futreal A, Tirabosco R, Flanagan AM. IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromasi but not in other mesenchymal tumours. J Pathol. 2011;224:334–43.

    Article  CAS  Google Scholar 

  5. Kato Kaneko M, Liu X, Oki H, Ogasawara S, Nakamura T, Saidoh N, Tsujimoto Y, Matsuyama Y, Uruno A, Sugawara M, Tsuchiya T, Yamakawa M, Yamamoto M, et al. Isocitrate dehydrogenase mutation is frequently observed in giant cell tumor of bone. Cancer Sci. 2014;105:744–8.

    Article  CAS  Google Scholar 

  6. Duncan CG, Barwick BG, Jin G, Rago C, Kapoor-Vazirani P, Powell DR, Jen-Tsan Chi J-T, Bigner DD, Vertino PM, Yan H. A heterozygous IDH1R132H/WT mutation induces genome-wide alterations in DNA methylation. Genome Res. 2012;22:2339–55.

    Article  CAS  Google Scholar 

  7. Turcan S, Rohle D, Goenka A, Walsh LA, Fang F, Yilmaz E, Campos C, Fabius AW, Lu C, Ward PS, Thompson CB, Kaufman A, Guryanova O, Levine R, Heguy A, Viale A, Morris LG, Huse JT, Mellinghoff IK, Chan TA. IDH1 mutation is sufficient to establish the glioma hypermethylator phenotype. Nature. 2012;483:479–83.

    Article  CAS  Google Scholar 

  8. Andronesi OC, Rapalino O, Gerstner E, Chi A, Batchelor TT, Cahill DP, Sorensen AG, Rosen BR. Detection of oncogenic IDH1 mutations using magnetic resonance spectroscopy of 2-hydroxyglutarate. J Clin Invest. 2013;123:3659–63.

    Article  CAS  Google Scholar 

  9. Dinardo CD, Propert KJ, Loren AW, Paietta E, Sun Z, Levine RL, Straley KS, Yen K, Patel JP, Agresta S, et al. Serum 2-hydroxyglutarate levels predict isocitrate dehydrogenase mutations and clinical outcome in acute myeloid leukemia. Blood. 2013;121:4917–24.

    Article  CAS  Google Scholar 

  10. Stein EM, DiNardo CD, Pollyea DA, Fathi AT, Roboz GJ, Altman JK, Stone RM, DeAngelo DJ, Levine RL, Flinn IW, et al. Enasidenib in mutant IDH2 relapsed or refractory acute myeloid leukemia. Blood. 2017;130:722–31.

    Article  CAS  Google Scholar 

  11. Szuhai K, Jennes I, de Jong D, Bovée JV, Wiweger M, Wuyts W, Hogendoorn PC. Tiling resolution array-CGH shows that somatic mosaic deletion of the EXT gene is causative in EXT gene mutation negative multiple osteochondromas patients. Hum Mutat. 2011;32:E2036–49.

    Article  CAS  Google Scholar 

  12. Reijnders CM, Waaijer CJ, Hamilton A, Buddingh EP, Dijkstra SP, Ham J, Bakker E, Szuhai K, Karperien M, Hogendoorn PC, Stringer SE, Bovée JV. No haploinsufficiency but loss of heterozygosity for EXT in multiple osteochondromas. Am J Pathol. 2010;177:1946–57.

    Article  CAS  Google Scholar 

  13. De Andrea CE, Reijnders CM, Kroon HM, et al. Secondary peripheral chondrosarcoma evolving from osteochondroma as a result of outgrowth of cells with functional EXT. Oncogene. 2012;31:1095.

    Article  Google Scholar 

  14. Bovée JV, Hogendoorn PC, Wunder JS, Alman BA. Cartilage tumours and bone development: molecular pathology and possible therapeutic targets. Nat Rev Cancer. 2010;10:481–8.

    Article  Google Scholar 

  15. Bovée JV, Cleton-Jansen AM, Taminiau AH, Hogendoorn PC. Emerging pathways in the development of chondrosarcoma of bone and implications for targeted treatment. Lancet Oncol. 2005;6:599–607.

    Article  Google Scholar 

  16. Tiet TD, Hopyan S, Nadesan P, Gokgoz N, Poon R, Lin AC, Yan T, Andrulis IL, Alman BA, Wunder JS. Constitutive hedgehog signaling in chondrosarcoma up-regulates tumor cell proliferation. Am J Pathol. 2006;168:321–30.

    Article  CAS  Google Scholar 

  17. Schrage YM, Briaire-de Bruijn IH, de Miranda NFCC, et al. Kinome profiling of chondrosarcoma reveals Src-pathway activity and dasatinib as option for treatment. Cancer Res. 2009;69:6216–22.

    Article  CAS  Google Scholar 

  18. Schrage YM, Machado I, Meijer D, Briaire-de Bruijn I, van den Akker BE, Taminiau AH, Kalinski T, Llombart-Bosch A, Bovée JV. COX-2 expression in chondrosarcoma: a role for celecoxib treatment? Eur J Cancer. 2010;46:616–24.

    Article  CAS  Google Scholar 

  19. van Oosterwijk JG, Anninga JK, Gelderblom H, Cleton-Jansen A-M, Bovée JVMG. Update on targets and novel treatment options for high-grade osteosarcoma and chondrosarcoma. Hematol Oncol Clin N Am. 2013;27:1021–48.

    Article  Google Scholar 

  20. van Oosterwijk JG, Herpers B, Meijer D, Briaire-de Bruijn IH, Cleton-Jansen AM, Gelderblom H, van de Water B, Bovée JV. Restoration of chemosensitivity for doxorubicin and cisplatin in chondrosarcoma in vitro: BCL-2 family members cause chemoresistance. Ann Oncol. 2012;23:1617–26.

    Article  Google Scholar 

  21. Schuetze SM, Bolejack V, Choy E, Ganjoo KN, Staddon AP, Chow WA, Tawbi HA, Samuels BL, Patel SR, von Mehren M, D’Amato G, Leu KM, Loeb DM, Forscher CA, Milhem MM, Rushing DA, Lucas DR, Chugh R, Reinke DK, Baker LH. Phase 2 study of dasatinib in patients with alveolar soft part sarcoma, chondrosarcoma, chordoma, epithelioid sarcoma, or solitary fibrous tumor. Cancer. 2017;123:90–7.

    Article  CAS  Google Scholar 

  22. Grignani G, Palmerini E, Stacchiotti S, Boglione A, Ferraresi V, Frustaci S, Comandone A, Casali PG, Ferrari S, Aglietta M. A phase 2 trial of imatinib mesylate in patients with recurrent nonresectable chondrosarcomas expressing platelet-derived growth factor receptor-alpha or -beta: an Italian Sarcoma Group Study. Cancer. 2011;117:826–31.

    Article  CAS  Google Scholar 

  23. Polychronidou G, Karavasilis V, Pollack SM, Huang PH, Lee A, Jones RL. Novel therapeutic approaches in chondrosarcoma. Future Oncol. 2017;13:637–48.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Laboratory of Experimental Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Maria Serena Benassi

Authors
  1. Maria Serena Benassi
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Editor information

Editors and Affiliations

  1. I.S.G. Italian Sarcoma Group, Bologna, Italy

    Piero Picci

  2. IIIrd Clinic of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy

    Marco Manfrini

  3. Istituto Ortopedico Rizzoli, Bologna, Italy

    Davide Maria Donati

  4. Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Marco Gambarotti

  5. Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Alberto Righi

  6. Istituto Rizzoli, Bologna, Italy

    Daniel Vanel

  7. Department of Medicine, University of Padua School of Medicine, Padua, Italy

    Angelo Paolo Dei Tos

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Benassi, M.S. (2020). Biology of Conventional Chondrosarcoma. In: Picci, P., et al. Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions. Springer, Cham. https://doi.org/10.1007/978-3-030-29676-6_35

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  • DOI: https://doi.org/10.1007/978-3-030-29676-6_35

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-29675-9

  • Online ISBN: 978-3-030-29676-6

  • eBook Packages: MedicineMedicine (R0)

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