Skip to main content

The Medical Management of Sarcomas

  • Chapter
  • First Online:
Bone Pathology
  • 3185 Accesses

Abstract

Sarcomas can be classified into those usually found in children and adolescents (osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma), and the non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) found primarily in adults. Chemotherapy and multidisciplinary care are essential for cure in the first group, while management of the second group is controversial. Evaluation involves imaging of primary and possible metastatic sites, biopsy and diagnosis (often using molecular tools), and marrow evaluation (in Ewing sarcoma and rhabdomyosarcoma). Most children with sarcomas are entered on clinical trials, with chemotherapy to prevent the emergence of metastases. Neo-adjuvant chemotherapy has become the standard approach to osteosarcoma, Ewing sarcoma, and most rhabdomyosarcomas. Primary tumor treatment is surgical in osteosarcomas, but may include surgery, radiation, or both in rhabdomyosarcoma and Ewing sarcoma. About two-thirds of patients with localized tumors are cured with current approaches, but the outlook for patients with metastases is much poorer. The role of chemotherapy in the treatment of patients with NRSTS is uncertain, and practice varies widely. Treatment of sarcomas has many acute toxic effects, as well as many late effects including second malignant neoplasms, growth disturbances, reduced fertility, and organ dysfunction.

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 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Aoki, J., H. Watanabe, et al (2001). “FDG PET of primary benign and malignant bone tumors: standardized uptake value in 52 lesions.” Radiology 219(3): 774–7.

    PubMed  CAS  Google Scholar 

  2. Franzius, C., J. Sciuk, et al (2000). “FDG-PET for detection of osseous metastases from malignant primary bone tumours: comparison with bone scintigraphy.” European Journal of Nuclear Medicine 27(9): 1305–11.

    Article  PubMed  CAS  Google Scholar 

  3. Franzius, C., H. E. Daldrup-Link, et al (2001). “FDG-PET for detection of pulmonary metastases from malignant primary bone tumors: comparison with spiral CT.” Annals of Oncology 12(4): 479–86.

    Article  PubMed  CAS  Google Scholar 

  4. Volker, T., T. Denecke, et al. (2007) “Positron-Emission Tomography for staging of pediatric sarcoma patients: Results of a prospective multicenter trial.” Journal of Clinical Oncology 34(25): 5435-5441.

    Article  Google Scholar 

  5. Mentzel H.-J., K. Kentouche, et al. (2004). “Comparison of while-body STIR-MRI and 99mTc-methylene-diphosphonate scintigraphy in children with suspected multifocal bone lesions.” European Radiology 14:2297–2302

    Article  PubMed  Google Scholar 

  6. Daldrup-Link, H. E., C. Franzius, et al (2001). “Whole-body MR imaging for detection of bone metastases in children and young adults: comparison with skeletal scintigraphy and FDG PET.” AJR American Journal of Roentgenology 177(1): 229–36.

    PubMed  CAS  Google Scholar 

  7. Lange, B. and A. S. Levine (1982). “Is it ethical not to conduct a prospectively controlled trial of adjuvant chemotherapy in osteosarcoma?” Cancer Treatment Reports 66(9): 1699–1703.

    PubMed  CAS  Google Scholar 

  8. Link, M. P., A. M. Goorin, et al (1986). “The Effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity.” New England Journal of Medicine 314(25): 1600–6.

    Article  PubMed  CAS  Google Scholar 

  9. Goorin, A. M., D. J. Schwartzentruber, et al (2003). “Presurgical chemotherapy compared with immediate surgery and adjuvant chemotherapy for nonmetastatic osteosarcoma: Pediatric Oncology Group Study POG-8651.[see comment].” Journal of Clinical Oncology 21(8): 1574–80.

    Article  PubMed  CAS  Google Scholar 

  10. Meyers P.A., C. Schwartz, et al. (2008) “Osteosarcoma: The addition of muramyl tripeptide to chemotherapy improves overall survival—A Children’s Oncology Group study. Journal of Clinical Oncology 26:633-638.

    Article  PubMed  CAS  Google Scholar 

  11. Nesbit, M. E., E. A. Gehan, et al (1990). “Multimodal therapy for the management of primary, nonmetastatic Ewing’s sarcoma of bone: a long-term follow-up of the first intergroup study.” Journal of Clinical Oncology 8(10 October): 1664–1674.

    PubMed  Google Scholar 

  12. Burgert, E. O., M. E. Nesbit, et al (1990). “Multimodal therapy for the management of nonpelvic localized Ewing’s sarcoma of bone: intergroup study IESS-II.” Journal of Clinical Oncology 8(9 September): 1514–1524.

    PubMed  Google Scholar 

  13. Grier, H. E., M. Krailo, et al (2003). “Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone.” New England Journal of Medicine 348(8): 694–701.

    Article  PubMed  CAS  Google Scholar 

  14. Granowetter, L., R.B. Womer, et al. (2009). “Dose-intensified compared with standard chemotherapy for nonmetastatic Ewing sarcoma family of tumors: a Children’s Oncology Group Study.” Journal of Clinical Oncology 27: 2536–2541.

    Article  PubMed  CAS  Google Scholar 

  15. Womer R.B., D.C. West, et al. (2008) “Randomized comparison of every-two-week v every-three-week chemotherapy in Ewing sarcoma family tumors (ESFT)” Journal of Clinical Oncology 26 (Suppl.), No. 10504.

    Google Scholar 

  16. Meyers, P. A., G. Heller, et al (1993). “Osteogenic sarcoma with clinically detectable metastasis at initial presentation.” Journal of Clinical Oncology 11(3): 449–53

    PubMed  CAS  Google Scholar 

  17. Kager, L., A. Zoubek, et al (2003). “Primary metastatic osteosarcoma: presentation and outcome of patients treated on neoadjuvant Cooperative Osteosarcoma Study Group protocols.” Journal of Clinical Oncology 21(10): 2011–8.

    Article  PubMed  Google Scholar 

  18. Miser, J., M. Krailo, et al (2004). “Treatment of metastatic Ewing’s sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide—a Children’s Cancer Group and Pediatric Oncology Group study.” Journal of Clinical Oncology 22(14): 2873–2877.

    Article  PubMed  CAS  Google Scholar 

  19. Marina, N. and P. Meyers (2005). “High-dose therapy and stem-cell rescue for Ewing’s family of tumors in second remission.” Journal of Clinical Oncology 23(19): 4262–4264.

    Article  PubMed  Google Scholar 

  20. Meyers, P. A., G. Heller, et al (1992). “Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience.” Journal of Clinical Oncology 10(1): 5–15.

    PubMed  CAS  Google Scholar 

  21. Bielack, S. S., B. Kempf-Bielack, et al (2002). “Prognostic factors in high-grade osteosarcoma of the extremities or trunk: an analysis of 1,702 patients treated on neoadjuvant cooperative osteosarcoma study group protocols.[see comment].” Journal of Clinical Oncology 20(3): 776–90.

    Article  PubMed  Google Scholar 

  22. Meyers, P. A., C. L. Schwartz, et al (2005). “Osteosarcoma: a randomized, prospective trial of the addition of ifosfamide and/or muramyl tripeptide to cisplatin, doxorubicin, and high-dose methotrexate.[see comment].” Journal of Clinical Oncology 23(9): 2004–11.

    Article  PubMed  CAS  Google Scholar 

  23. Barr, F. G., S. J. Qualman, et al (2002). “Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions.” Cancer Research 62(16): 4704–10.

    PubMed  CAS  Google Scholar 

  24. Rodary, C., E. A. Gehan, et al (1991). “Prognostic factors in 951 nonmetastatic rhabdomyosarcoma in children: a report from the international rhabdomyosarcoma workshop.” Medical and Pediatric Oncology 19: 89–95.

    Article  PubMed  CAS  Google Scholar 

  25. Heyn, R. M., R. Holland, et al (1974). “The role of combined chemotherapy in the treatment of rhabdomyosarcoma in children.” Cancer 34: 2128–2142.

    Article  PubMed  CAS  Google Scholar 

  26. Arndt C.A., D.S. Hawkins, et al. (2007) “Randomized phase III trial comparing vincristine, actinomycin, cyclophosphamide (VAC) with VAC/V topotecan/cyclophosphamide (TC) for intermediate risk rhabdomyosarcoma (IRRMS). D9803, COG study.” Journal of Clinical Oncology 25(18 Suppl.), No. 9509.

    Google Scholar 

  27. Ortega, J. A. R. J et al, (2000). “Presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma: implications for outcome”. Journal Pediatric Hematology/Oncology 22(2): 106–111.

    Article  CAS  Google Scholar 

  28. Pratt, C., A. Pappo, et al (1999). “Role of adjuvant chemotherapy in the treatment of surgically resected pediatric nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group study.” Journal of Clinical Oncology 7: 1219–1226.

    Google Scholar 

  29. Okcu, M. F., M. Munsell, et al (2003). “Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome.” Journal of Clinical Oncology 21(8): 1602–11.

    Article  PubMed  Google Scholar 

  30. Grobmyer, S. R., R. G. Maki, et al (2004). “Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma.” Annals of Oncology 15(11): 1667–72.

    Article  PubMed  CAS  Google Scholar 

  31. Oeffinger K.C., A.C. Mertens, et al. (2006) “Chronic health conditions in adult survivors of childhood cancer.” New England Journal of Medicine 355(15):1572–1582.

    Article  PubMed  CAS  Google Scholar 

  32. Tucker, M. A., G. J. D’Angio, et al (1987). “Bone sarcomas linked to radiotherapy and chemotherapy in children.” The New England Journal of Medicine 317: 588–593.

    Article  PubMed  CAS  Google Scholar 

  33. Neglia, J. P., D. L. Friedman, et al (2001). “Second malignant neoplasms in five-year survivors of childhood cancer: childhood cancer survivor study.” Journal of the National Cancer Institute 93(8): 618–29.

    Article  PubMed  CAS  Google Scholar 

  34. Kuttesch, J. F. J., L. H. Wexler, et al (1996). “Second malignancies after Ewing’s sarcoma: radiation dose-dependency of secondary sarcomas.” Journal of Clinical Oncology 14(10 (October)): 2818–2825.

    PubMed  Google Scholar 

  35. Bassal M., A.C. Mertens, et al. (2006) “Risk of selected subsequent carcinomas in survivors of childhood cancer: A report from the Childhood Cancer Survivor Study.” Journal of Clinical Oncology 24:476–483.

    Article  PubMed  Google Scholar 

  36. Li, Y., R. B. Womer, et al (2004). “Predicting cisplatin ototoxicity in children: the influence of age and the cumulative dose.[see comment].” European Journal of Cancer 40(16): 2445–51.

    Article  PubMed  CAS  Google Scholar 

  37. Berenson, J. R. (2005). “Recommendations for zoledronic acid treatment of patients with bone metastases.” Oncologist 10(1): 52–62.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Richard B. Womer .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Humana Press, a part of Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Womer, R.B. (2009). The Medical Management of Sarcomas. In: Khurana, J. (eds) Bone Pathology. Humana Press. https://doi.org/10.1007/978-1-59745-347-9_17

Download citation

  • DOI: https://doi.org/10.1007/978-1-59745-347-9_17

  • Published:

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-766-2

  • Online ISBN: 978-1-59745-347-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics