Skip to main content
SpringerLink
Log in

Musculoskeletal Tumors (Osteosarcoma and Ewing’s Sarcoma)

  • Living reference work entry
  • First Online:
Pediatric Surgery

  • 56 Accesses

Abstract

Non-rhabdomyosarcoma soft tissue sarcomas are rare heterogeneous tumors. In a total of more than 50 histologies, they are more common than rhabdomyosarcoma. Here we focus on the most common types of bone and soft tissue sarcoma in children. A summary of diagnosis and treatment is included, with a surgical focus. The most recent treatment strategies are emphasized with evidence-based approach.

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

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Bacci G, Picci P, et al. No advantages in the addition of ifosfamide and VP-16 to the standard four-drug regimen in the maintenance phase of neoadjuvant chemotherapy of Ewing’s sarcoma of bone: results of two sequential studies. J Chemother. 1993;5(4):247–57.

    Article  CAS  PubMed  Google Scholar 

  • Black JO, Coffin C, Anderson J Hawkins D, McCarville ME, Kao SC, Randall RL, Hayes-Jordan A, Parham D, Spunt S. Nonrhabdomyosarcoma myosarcoma soft tissue sarcomas in children, adolescents, and young adults: overview of COG ARST0332, A report from the Children’s Oncology Group. 2013 CTOS Annual Meeting, New York. 2013.

    Google Scholar 

  • Briccoli A, Rocca M, et al. High grade osteosarcoma of the extremities metastatic to the lung: long-term results in 323 patients treated combining surgery and chemotherapy, 1985–2005. Surg Oncol. 2010;19(4):193–9.

    Article  PubMed  Google Scholar 

  • Burdach S, Jurgens H, et al. Myeloablative radiochemotherapy and hematopoietic stem-cell rescue in poor-prognosis Ewing’s sarcoma. J Clin Oncol. 1993;11(8):1482–8.

    Article  CAS  PubMed  Google Scholar 

  • Clark J, Rocques PJ, et al. Identification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcoma. Nat Genet. 1994;7(4):502–8.

    Article  CAS  PubMed  Google Scholar 

  • Cortes EP, Holland JF, et al. Amputation and adriamycin in primary osteosarcoma. N Engl J Med. 1974;291(19):998–1000.

    Article  CAS  PubMed  Google Scholar 

  • Ferrari A, Casanova M. New concepts for the treatment of pediatric on rhabdomyosarcoma soft tissue sarcomas. Expert Rev Anticancer Ther. 2005;5(2):307–18.

    Article  CAS  PubMed  Google Scholar 

  • Fisher C. The diversity of soft tissue tumours with EWSR1 gene rearrangements: a review. Histopathology. 2014;64(1):134–50.

    Article  PubMed  Google Scholar 

  • Goorin AM, Frei E 3rd, et al. Adjuvant chemotherapy for osteosarcoma: a decade of experience. Surg Clin North Am. 1981;61(6):1379–89.

    Article  CAS  PubMed  Google Scholar 

  • Goorin AM, Abelson HT, et al. Osteosarcoma: fifteen years later. N Engl J Med. 1985;313(26):1637–43.

    Article  CAS  PubMed  Google Scholar 

  • Gorlick R, Janeway K, et al. Children’s Oncology Group’s 2013 blueprint for research: bone tumors. Pediatr Blood Cancer. 2013;60(6):1009–15.

    Article  PubMed  Google Scholar 

  • Gurney JG, Severson RK, et al. Incidence of cancer in children in the United States. Sex-, race-, and 1-year age-specific rates by histologic type. Cancer. 1995;75(8):2186–95.

    Article  CAS  PubMed  Google Scholar 

  • Harting MT, Blakely ML, et al. Long-term survival after aggressive resection of pulmonary metastases among children and adolescents with osteosarcoma. J Pediatr Surg. 2006;41(1):194–9.

    Article  PubMed  Google Scholar 

  • Hawkins D, Spunt SL, Skapek SX, on behalf of the COG Soft Tissue Sarcoma Committee. Children’s Oncology Group’s 2013 blueprint for research: soft tissue sarcomas. Pediatr Blood Cancer. 2013;60(6):1001–8.

    Article  PubMed  Google Scholar 

  • Hayes-Jordan A, Anderson P. Desmoplastic small cell round tumor: review of therapy including surgery followed by continuous hyperthermic peritoneal perfusion of chemotherapy. Oncol Rev. 2009;3(3):195–200.

    Article  Google Scholar 

  • Hayes-Jordan AA, Spunt SL, et al. Nonrhabdomyosarcoma soft tissue sarcomas in children: is age at diagnosis an important variable? J Pediatr Surg. 2000;35(6):948–53; discussion 953–944.

    Article  CAS  PubMed  Google Scholar 

  • Hayes-Jordan A, Anderson P, et al. Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor. J Pediatr Surg. 2007;42(8):E29–32.

    Article  PubMed  Google Scholar 

  • Hayes-Jordan A, Green H, et al. Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion. J Pediatr Surg. 2010;45(5):1000–6.

    Article  PubMed  Google Scholar 

  • Hayes-Jordan A, Green HL, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol. 2014;21(1):220–4.

    Article  PubMed  Google Scholar 

  • Horowitz ME, Kinsella TJ, et al. Total-body irradiation and autologous bone marrow transplant in the treatment of high-risk Ewing’s sarcoma and rhabdomyosarcoma. J Clin Oncol. 1993;11(10):1911–8.

    Article  CAS  PubMed  Google Scholar 

  • Huh WW, Fitzgerald NE, et al. Peritoneal sarcomatosis in pediatric malignancies. Pediatr Blood Cancer. 2013a;60(1):12–7.

    Google Scholar 

  • Huh WW, Wang W-L, Lazar A, Daw NC, Herzog CE, Hughes DPM, Anderson PM, Munsell MF, McAleer MF, Hayes-Jordan A. Undifferentiated soft-tissue sarcoma in children and young adults: the MD Anderson Cancer Center experience. 2012 CTOS 17th Annual Meeting, Prague, Czech Republic. 2013b.

    Google Scholar 

  • Jaffe N, Goorin A, et al. High-dose methotrexate in osteogenic sarcoma adjuvant chemotherapy and limb salvage results. Cancer Treat Rep. 1981;65(Suppl 1):99–106.

    PubMed  Google Scholar 

  • Kushner BH, LaQuaglia MP, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol. 1996;14(5):1526–31.

    Article  CAS  PubMed  Google Scholar 

  • Ladanyi M, Gerald W. Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor. Cancer Res. 1994;54(11):2837–40.

    CAS  PubMed  Google Scholar 

  • Lal DR, Su WT, et al. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005;40(1):251–5.

    Article  PubMed  Google Scholar 

  • Leary SE, Wozniak AW, et al. Survival of pediatric patients after relapsed osteosarcoma: the St. Jude Children’s Research Hospital experience. Cancer. 2013;119(14):2645–53.

    Article  CAS  PubMed  Google Scholar 

  • Lee MJ, Chhabra A, Pressey JG, et al. MR imaging of pediatric musculoskeletal tumors: recent advances and clinical applications. Magn Reson Imaging Clin N Am. 2019;27(2):341–71.

    Article  PubMed  Google Scholar 

  • Letourneau PA, Shackett B, et al. Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival. J Pediatr Surg. 2011a;46(2):332–5.

    Article  PubMed  PubMed Central  Google Scholar 

  • Letourneau PA, Xiao L, et al. Location of pulmonary metastasis in pediatric osteosarcoma is predictive of outcome. J Pediatr Surg. 2011b;46(7):1333–7.

    Article  PubMed  PubMed Central  Google Scholar 

  • Luksch R, Tienghi A, et al. Primary metastatic Ewing’s family tumors: results of the Italian Sarcoma Group and Scandinavian Sarcoma Group ISG/SSG IV Study including myeloablative chemotherapy and total-lung irradiation. Ann Oncol. 2012;23(11):2970–6.

    Article  CAS  PubMed  Google Scholar 

  • Picci P, Vanel D, et al. Computed tomography of pulmonary metastases from osteosarcoma: the less poor technique. A study of 51 patients with histological correlation. Ann Oncol. 2001;12(11):1601–4.

    Article  CAS  PubMed  Google Scholar 

  • Qureshi SS, Kembhavi S, et al. Prognostic factors in primary nonmetastatic Ewing sarcoma of the rib in children and young adults. J Pediatr Surg. 2013;48(4):764–70.

    Article  PubMed  Google Scholar 

  • Rao AD, Chen Q, Million L, et al. Preoperative intensity modulated radiation therapy compared to three-dimensional conformal radiation therapy for high-grade extremity sarcomas in children: analysis of the Children’s Oncology Group Study ARST0332. Int J Radiat Oncol Biol Phys. 2019;103(1):38–44.

    Article  PubMed  Google Scholar 

  • Rasalkar DD, Chu WC, et al. Pulmonary metastases in children with osteosarcoma: characteristics and impact on patient survival. Pediatr Radiol. 2011;41(2):227–36.

    Article  PubMed  Google Scholar 

  • Rodeberg D, Arndt C, et al. Characteristics and outcomes of rhabdomyosarcoma patients with isolated lung metastases from IRS-IV. J Pediatr Surg. 2005;40(1):256–62.

    Article  PubMed  Google Scholar 

  • Schofield DE, Fletcher JA, et al. Fibrosarcoma in infants and children. Application of new techniques. Am J Surg Pathol. 1994;18(1):14–24.

    Article  CAS  PubMed  Google Scholar 

  • Sheng WQ, Hisaoka M, et al. Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. Am J Clin Pathol. 2001;115(3):348–55.

    Article  CAS  PubMed  Google Scholar 

  • Simon MA, Springfield DS. Surgery for bone and soft-tissue tumors. Philadelphia: Lippincott-Raven Publishers; 1998.

    Google Scholar 

  • Spunt SL, Poquette CA, et al. Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children’s Research Hospital. J Clin Oncol. 1999;17(12):3697–705.

    Article  CAS  PubMed  Google Scholar 

  • Spunt SL, Hill DA, et al. Clinical features and outcome of initially unresected nonmetastatic pediatric nonrhabdomyosarcoma soft tissue sarcoma. J Clin Oncol. 2002;20(15):3225–35.

    Article  PubMed  Google Scholar 

  • Strehl S, Ladenstein R, et al. Translocation (12;13) in a case of infantile fibrosarcoma. Cancer Genet Cytogenet. 1993;71(1):94–6.

    Article  CAS  PubMed  Google Scholar 

  • Szegezdi E, O'Reilly A, et al. Stem cells are resistant to TRAIL receptor-mediated apoptosis. J Cell Mol Med. 2009;13(11–12):4409–14.

    Article  CAS  PubMed  Google Scholar 

  • Tognon C, Garnett M, et al. The chimeric protein tyrosine kinase ETV6-NTRK3 requires both Ras-Erk1/2 and PI3-kinase-Akt signaling for fibroblast transformation. Cancer Res. 2001;61(24):8909–16.

    CAS  PubMed  Google Scholar 

  • Wai DH, Knezevich SR, et al. The ETV6-NTRK3 gene fusion encodes a chimeric protein tyrosine kinase that transforms NIH3T3 cells. Oncogene. 2000;19(7):906–15.

    Article  CAS  PubMed  Google Scholar 

  • Wexler LH, DeLaney TF, et al. Ifosfamide and etoposide plus vincristine, doxorubicin, and cyclophosphamide for newly diagnosed Ewing’s sarcoma family of tumors. Cancer. 1996;78(4):901–11.

    Article  CAS  PubMed  Google Scholar 

  • Wu PK, Chen WM, et al. Primary osteogenic sarcoma with pulmonary metastasis: clinical results and prognostic factors in 91 patients. Jpn J Clin Oncol. 2009;39(8):514–22.

    Article  PubMed  Google Scholar 

  • Young JL Jr, Ries LG, et al. Cancer incidence, survival, and mortality for children younger than age 15 years. Cancer. 1986;58(2 Suppl):598–602.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Andrea A. Hayes-Jordan

  2. University of North Carolina School of Medicine Chapel Hill, North Carolina, USA

    Andrea A. Hayes-Jordan

  3. Department of Orthopaedic Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Valerae O. Lewis

Authors
  1. Andrea A. Hayes-Jordan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Valerae O. Lewis
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andrea A. Hayes-Jordan .

Editor information

Editors and Affiliations

  1. Our Lady’s Children's Hospital, National Children’s Research Centre, Dublin, Ireland

    Prem Puri

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer-Verlag GmbH Germany, part of Springer Nature

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Hayes-Jordan, A.A., Lewis, V.O. (2020). Musculoskeletal Tumors (Osteosarcoma and Ewing’s Sarcoma). In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_150-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-38482-0_150-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-38482-0

  • Online ISBN: 978-3-642-38482-0

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation