Ewing Sarcoma and Primitive Neuroectodermal Tumors Workup

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

Laboratory Studies

  • No diagnostic blood studies provide pathognomonic or suggestive results to diagnose ESFT.
  • Depending on the patient’s age and presenting symptoms, blood tests might be helpful in evaluating other diagnoses. Such tests may include blood cultures, measurement of C-reactive protein levels, and determinations of the CBC count and erythrocyte sedimentation rate.
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Imaging Studies

  • Evaluation of the primary lesion
    • The priority is to obtain images of the suspected primary lesion or of any region with symptoms.
    • If a bony mass is palpated, plain radiography is indicated.
    • MRI of the region can help in determining the extent of disease. MRI is immediately required if tumors are adjacent to critical neurologic structures, and emergency radiation therapy, surgery, and/or steroids should be considered to prevent nerve damage.
    • CT imaging is helpful to delineate any bony involvement.
  • Evaluation for metastases
    • Metastatic evaluation includes chest CT and radioisotope bone scanning.
    • If the initial results suggest that a tumor is likely, chest CT scanning should be performed before surgical biopsy to avoid confusion of this finding with postoperative atelectasis.
    • Most centers now use whole-body body MRI or fluorodeoxyglucose (FDG) positron emission tomography (PET) as sensitive tools to detect metastatic disease. Neither modality is associated with an improved prognosis. However, a general consensus among investigators suggests that localized disease may occur at distal sites because metastatic disease is underdiagnosed, among other reasons.[1]
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Procedures

  • Biopsy
    • If a lesion of the Ewing sarcoma family of tumors or another tumor is probable, consultation with a pediatric oncologist should be sought before a biopsy is performed.
    • A biopsy specimen is required for definitive diagnosis.
    • The biopsy specimen should be evaluated by means of routine staining as well as immunohistochemical analysis with antibodies to differentiate the lesion from other small round blue cell tumors, such as rhabdomyosarcoma and lymphoma.
    • Biopsy should be performed after any potential therapy is fully considered because all patients with tumors of the Ewing sarcoma family require some form of definitive local treatment.
    • Inappropriate biopsy or resection often increases patient morbidity or mortality. An example is a biopsy incision that extends outside the tumor resection at the time of definitive surgery. This causes the surgeon to excise additional tumor-contaminated tissue that might have been spared if proper planning occurred prior to a biopsy.
  • Cytogenetic and molecular studies
    • Cytogenetic studies should be used to confirm the diagnosis if t(11;22) or a related translocation is found.
    • For standard cytogenetics, fresh tissue should be sent in appropriate media to a cytogenetic laboratory.
    • In addition, a small piece of the tumor should be snap frozen in liquid nitrogen for molecular studies.
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Histologic Findings

  • Tumors of the Ewing sarcoma family are small, round, blue cell tumors. They can be undifferentiated or differentiated, as reflected in rosette formation.
  • Immunohistochemical markers include membranous staining with MIC2 (12E7) antigen (CD99), which is characteristic but not pathognomonic. Muscle, lymphoid, and adrenergic markers should be negative.
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Staging

  • Staging includes both local imaging to reveal the full extent of tumor prior to therapy and evaluation for distant metastases.
  • Local imaging usually includes both MRI and CT scanning (see Imaging Studies). When bone is involved, these are complimentary techniques. For soft-tissue lesions, MRI should be adequate in most cases.
  • The evaluation for metastases should include bilateral bone marrow biopsies (some centers obtain multiple cores on each side, but this is not well supported), chest CT scanning, and radionuclide total body scanning, such as technetium-99 scanning (see Procedures). Many centers are now using FDG-PET scanning or total body MRI to look for occult metastases. Although these techniques often produce false-positive results that require biopsy, some findings suggest that locating these occult metastases and providing local therapy (radiation or surgery) improves survival.
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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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