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Key Features

Essentials of Diagnosis

  • The cardinal signs of bone tumor are pain at the site of involvement, often following slight trauma, mass formation, and fracture through an area of cortical bone destruction

  • Second most common malignant tumor of bony origin

  • Although most commonly a tumor of bone, it may also occur in soft tissue (extraosseous Ewing sarcoma or peripheral neuroectodermal tumor [PNET])

General Considerations

  • Considered a "small, round, blue cell" malignancy

  • Accounts for only 30% of primary malignant bone tumors

  • Fewer than 200 new cases occur each year in the United States

  • Primarily affects white males, almost never affects blacks

  • Occurs in toddlers to young adults

Clinical Findings

Symptoms and Signs

  • Pain at the site of the primary tumor is the most common presenting sign, with or without swelling and erythema

  • Associated symptoms include fevers and weight loss

Differential Diagnosis

  • Rhabdomyosarcoma

  • Lymphoma

  • Neuroblastoma


Laboratory Findings

  • No specific laboratory findings are characteristic

  • However, an elevated lactate dehydrogenase may be present

Imaging and Staging

  • Radiographs show diaphyses of long bones

  • MRI of the primary lesion should be done to define the extent of local disease as precisely as possible

  • CT scan of the chest, bone scan, and bilateral bone marrow aspirates and biopsies are all essential to the staging workup

  • PET-CT may help monitor therapy response

Diagnostic Procedures

  • A biopsy is essential in establishing the diagnosis

  • Histologically, Ewing sarcoma consists of sheets of undifferentiated cells with hyperchromatic nuclei, well-defined cell borders, and scanty cytoplasm. Necrosis is common

  • Electron microscopy, immunocytochemistry, and cytogenetics may be necessary to confirm the diagnosis

  • A generous tissue biopsy specimen is often necessary for diagnosis but should not delay starting chemotherapy


  • Chemotherapy is started after biopsy and is followed by local control measures

    • Effective chemotherapy uses combinations of dactinomycin, vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide

    • Local measures include surgery, radiation therapy, or a combination of these methods

  • Following local control, chemotherapy continues for approximately 6 months



  • Patients with small localized primary tumors have a 70–75% long-term, disease-free survival rate

  • For patients with metastatic disease survival is poor

  • Patients with pelvic tumors have about a 50% long-term, disease-free survival


Geller  DS, Gorlick  R: Osteosarcoma: a review of diagnosis, management and treatment strategies. Clin Adv Hematol Oncol 2010;8(10):705
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Moore  DD  et al: Ewing sarcoma of bone. Cancer Treat Res 2014;162:93
[PubMed: 25070232]
Womer ...

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