Ewing Sarcoma and Primitive Neuroectodermal Tumors Clinical Presentation

  • Author: Jeffrey A Toretsky, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Nov 28, 2011
 

History

  • Patients usually present with pain.
  • Patients often have a palpable mass.
  • Patients with lesions of the long bones can present with a pathologic fracture.
  • Back pain may indicate a paraspinal, retroperitoneal, or deep pelvic tumor.
  • Systemic symptoms of fever and weight loss can also occur and often indicate metastatic disease.
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Physical

  • Tumors of the Ewing sarcoma family can occur in virtually any location. Careful examination of painful sites with inspection and palpation is critical.
  • Because patients can present with disease close to bone, tumors can result in neuropathic pain. Therefore, a comprehensive neurologic examination to evaluate asymmetric weakness, numbness, or pain is critical.
  • Patients with lung metastases can present with asymmetric breath sounds, pleural signs, or rales.
  • Patients with clinically significant bone marrow metastases can present with petechiae or purpura due to thrombocytopenia.
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Causes

  • The cause is unknown.
  • Cases are thought to be sporadic. However, the incidence of neuroectodermal and stomach malignancies is increased among family members of patients with tumors of the Ewing sarcoma family.
  • Ewing sarcoma family tumors are rarely reported after the treatment of another neoplasm (second malignancy).
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Contributor Information and Disclosures
Author

Jeffrey A Toretsky, MD  Associate Professor, Departments of Oncology and Pediatrics, Lombardi Comprehensive Cancer Center, Georgetown University School of Medicine

Disclosure: Georgetown Intellectual property rights Investigator

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

  1. Kim DH, Kim SY, Lee HJ, Song BS, Kim DH, Cho JB, et al. Assessment of Chemotherapy Response Using FDG-PET in Pediatric Bone Tumors: A Single Institution Experience. Cancer Res Treat. Sep 2011;43(3):170-5. [Medline]. [Full Text].

  2. Miser JS, Krailo MD, Tarbell NJ, et al. 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. J Clin Oncol. Jul 15 2004;22(14):2873-6. [Medline].

  3. Womer, West, Krailo, Pawel, Dickman. hemotherapy intensification by interval compression in localized Ewing Sarcoma Family Tumors. Seattle, WA: Connective Tissue Oncology Society Annual Meeting; 2007.

  4. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. Feb 20 2003;348(8):694-701. [Medline].

  5. Dunst J, Jurgens H, Sauer R, Pape H, Paulussen M, Winkelmann W, et al. Radiation therapy in Ewing's sarcoma: an update of the CESS 86 trial. Int J Radiat Oncol Biol Phys. Jul 15 1995;32(4):919-30. [Medline].

  6. Israelsen RB, Ilium BE, Crabtree S, Randall RL, Jones KB. Extremity sarcoma surgery in younger children: ten years of patients ten years and under. Iowa Orthop J. 2011;31:145-53. [Medline]. [Full Text].

  7. Gurney JG, Swensen AR, Bulterys M. Malignant bone tumors. In: Ries LA, Smith MAS, Gurney JG, et al, eds. Cancer Incidence and Survival Among Children and Adolescents: United States SEER Program 1975-1995. Publication 99-4649. Bethesda, MD: National Cancer Institute; 1999:99-110.

  8. Meyers PA, Krailo MD, Ladanyi M, Chan KW, Sailer SL, Dickman PS, et al. High-dose melphalan, etoposide, total-body irradiation, and autologous stem-cell reconstitution as consolidation therapy for high-risk Ewing's sarcoma does not improve prognosis. J Clin Oncol. Jun 1 2001;19(11):2812-20. [Medline].

  9. Paulussen M, Ahrens S, Dunst J, Winkelmann W, Exner GU, Kotz R, et al. Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86. J Clin Oncol. Mar 15 2001;19(6):1818-29. [Medline].

  10. Saylors RL 3rd, Stine KC, Sullivan J, et al. Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study. J Clin Oncol. Aug 1 2001;19(15):3463-9. [Medline].

  11. Uren A, Toretsky JA. Ewing's sarcoma oncoprotein EWS-FLI1: the perfect target without a therapeutic agent. Future Oncol. Aug 2005;1(4):521-8. [Medline].

 
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