Skip to main content

Abstract

Osteosarcomas are uncommon, accounting for only 1 % of all cancers diagnosed annually in the United States. The incidence peaks in early adolescence and in adults aged >65 years. This cancer has a predilection for the metaphyseal region of the long bones, and localized pain that lasts for several months is the most common symptom. The histologic subtypes include osteoblastic, fibroblastic, and chondroblastic. Less common variants include Ewing’s sarcoma, small cell, telangiectatic, multifocal, and a malignant fibrous histiocytoma subtype. The staging assessment includes magnetic resonance imaging, computed tomography, bone scan, and/or positron emission tomography. It usually spreads hematogenously, and the main site of metastasis is the lung.

It is necessary to consider limb preservation in patients with localized disease. The recommended regimens for non-metastatic disease include neoadjuvant and adjuvant cisplatin plus doxorubicin-based chemotherapy. In patients aged <30 years, adding high-dose methotrexate is reasonable. The mainstay treatment is tumor resection preferably with wide margins within 12 weeks after starting chemotherapy.

In metastatic disease limited to the lungs, it is important to consider resection. Recurrence is common, indicating second-line chemotherapy regimens containing ifosfamide, etoposide, and carboplatin.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Huvos A (1991) Bone tumors: diagnosis, treatment, prognosis, 2nd edn. WB Saunders, Philadelphia

    Google Scholar 

  2. Mirabello L, Troisi RJ, Savage SA (2009) Osteosarcoma incidence and survival rates from 1973 to 2004: data from the Surveillance, Epidemiology, and End Results Program. Cancer 115:1531

    Article  PubMed Central  PubMed  Google Scholar 

  3. Grimer RJ et al (2003) Eur J Cancer 39(2):157–163

    Article  PubMed  CAS  Google Scholar 

  4. Gupta S, Chitra S, Singh D (2008) Primary osteogenic sarcoma of skull bone-a rare clinical presentation. Internet J Oncol 6:2

    Google Scholar 

  5. Thorpe WP, Reilly JJ, Rosenberg SA (1979) Prognostic significance of alkaline phosphatase measurements in patients with osteogenic sarcoma receiving chemotherapy. Cancer 43:2178

    Article  PubMed  CAS  Google Scholar 

  6. Link MP, Goorin AM, Horowitz M, et al (1991) Adjuvant chemotherapy of high-grade osteosarcoma of the extremity. Updated results of the Multi-Institutional Osteosarcoma Study. Clin Orthop Relat Res (270):8–14

    Google Scholar 

  7. (2010) AJCC cancer staging manual, 7th edn. Springer, New York, USA

    Google Scholar 

  8. Inwards CY, Unni KK (1995) Classification and grading of bone sarcomas. Hematol Oncol Clin North Am 9(3):545–69

    PubMed  CAS  Google Scholar 

  9. Rosen G, Marcove RC, Huvos AG et al (1983) Primary osteogenic sarcoma: eight-year experience with adjuvant chemotherapy. J Cancer Res Clin Oncol 106(Suppl):55–67

    Article  PubMed  Google Scholar 

  10. Eilber F, Giuliano A, Eckardt J et al (1987) Adjuvant chemotherapy for osteosarcoma: a randomized prospective trial. J Clin Oncol 5:21

    PubMed  CAS  Google Scholar 

  11. Link MP, Goorin AM, Miser AW et al (1986) The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity. N Engl J Med 314:1600

    Article  PubMed  CAS  Google Scholar 

  12. Bernthal NM, Federman N, Eilber FR et al (2012) Long-term results (>25 years) of a randomized, prospective clinical trial evaluating chemotherapy in patients with high-grade, operable osteosarcoma. Cancer 118:5888

    Article  PubMed  CAS  Google Scholar 

  13. Daniel A, Christina LS et al (2012) A retrospective safety analysis of adult patients treated with high-dose methotrexate for osteosarcoma in Stockholm, Sweden. J Clin Oncol 30 (suppl; abstr 10083)

    Google Scholar 

  14. Souhami RL, Craft AW, Van der Eijken JW et al (1997) Randomised trial of two regimens of chemotherapy in operable osteosarcoma: a study of the European Osteosarcoma Intergroup. Lancet 350:911

    Article  PubMed  CAS  Google Scholar 

  15. Bramwell VH, Burgers M, Sneath R et al (1992) A comparison of two short intensive adjuvant chemotherapy regimens in operable osteosarcoma of limbs in children and young adults: the first study of the European Osteosarcoma Intergroup. J Clin Oncol 10:1579

    PubMed  CAS  Google Scholar 

  16. Gentet JC, Brunat-Mentigny M, Demaille MC et al (1997) Ifosfamide and etoposide in childhood osteosarcoma. A phase II study of the French Society of Paediatric Oncology. Eur J Cancer 33:232

    Article  PubMed  CAS  Google Scholar 

  17. Van Winkle P et al (2005) Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children’s Cancer Group (CCG) experience. Pediatr Blood Cancer 44(4):338–347

    Article  PubMed  Google Scholar 

  18. Navid F, Willert JR, McCarville MB et al (2008) Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma. Cancer 113:419

    Article  PubMed  CAS  Google Scholar 

  19. Grimer RJ, Bielack S, Flege S et al (2005) Periosteal osteosarcoma—a European review of outcome. Eur J Cancer 41:2806–2811

    Article  PubMed  Google Scholar 

  20. Jedlicka P (2010) Ewing sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by transcription factor oncogenic fusions. Int J Clin Exp Pathol 3:338

    PubMed Central  PubMed  CAS  Google Scholar 

  21. Fizazi K, Dohollou N, Blay JY et al (1998) Ewing’s family of tumors in adults: multivariate analysis of survival and long-term results of multimodality therapy in 182 patients. J Clin Oncol 16:3736

    PubMed  CAS  Google Scholar 

  22. Grier HE, Krailo MD, Tarbell NJ et al (2003) Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 348:694

    Article  PubMed  CAS  Google Scholar 

  23. Dunst J, Schuck A (2004) Role of radiotherapy in Ewing tumors. Pediatr Blood Cancer 42:465

    Article  PubMed  Google Scholar 

  24. Hogendoorn PCW, Bovee JM, Nielsen GP (2013) Chondrosarcoma (grades I-III), including primary and secondary variants and periosteal chondrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (eds) World Health Organization classification of tumours of soft tissue and bone, vol 5, 4th edn. IARC, Lyon, p 264

    Google Scholar 

  25. Murphey MD, Walker EA, Wilson AJ et al (2003) From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics 23:1245

    Article  PubMed  Google Scholar 

  26. Huvos AG, Heilweil M, Bretsky SS (1985) The pathology of malignant fibrous histiocytoma of bone. A study of 130 patients. Am J Surg Pathol 9(12):853–871

    Article  PubMed  CAS  Google Scholar 

  27. Werner M (2006) Giant cell tumour of bone: morphological, biological and histogenetical aspects. Int Orthop 30:484

    Article  PubMed Central  PubMed  Google Scholar 

  28. Leggon RE, Zlotecki R, Reith J, Scarborough MT (2004) Giant cell tumor of the pelvis and sacrum: 17 cases and analysis of the literature. Clin Orthop Relat Res 196

    Google Scholar 

  29. Stock GT, Teixeira LLC, Aguiar PN Jr, Jose FF, Ribas C (2014) Metastatic giant cell tumor of bone: a case report. J Med Cases 5(11):557–560

    Article  Google Scholar 

  30. Thomas D, Henshaw R, Skubitz K et al (2010) Denosumab in patients with giant-cell tumour of bone: an open-label, phase 2 study. Lancet Oncol 11:275

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Ramon Andrade de Mello M.D., Ph.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Vega, M.C.M.D., Aguiar, P.N., Tadokoro, H., de Mello, R.A. (2015). Bone Sarcomas. In: de Mello, R., Tavares, Á., Mountzios, G. (eds) International Manual of Oncology Practice. Springer, Cham. https://doi.org/10.1007/978-3-319-21683-6_30

Download citation

Publish with us

Policies and ethics