EWING’S SARCOME

James Ewing, 1921 Dr. James Ewing (1866 – 1943)

TIM IV IH,EG,DD,RM

Pediatric Bone “Tumors”
Benign
Osteochondroma  Osteoid Osteoma  Enchondroma  Chondroblastoma  Non-ossifying fibroma aka benign cortical defect  Hemangioma  Eosinophilic granuloma  Osteomyelitis

Malignant
Osteosarcoma  Ewing sarcoma  Malignant fibrous histiocytoma  Non-Hodgkin Lymphoma  Eosinophilic granuloma

Malignant bone tumors
  

Rare 6% of all childhood malignancies Annual US Incidence in children < 20 yrs
◦ 8.7 per million ~ 650 to 700 children/year

Most often occur in young patients < 25 yrs  Most common bone tumors

◦ Osteosarcoma ◦ Ewing sarcoma

56% 34%

Ewing Sarcoma (EWS)  Represents a family of tumors including ◦ Ewing sarcoma of bone ◦ extraosseous Ewing sarcoma and ◦ peripheral neuroectodermal tumor (PNET) of bone or soft tissue  2nd most common bone tumor in children .

 Rare in African-Americans and Chinese  ..o. females 5-9 y.Epidemiology 1% of all childhood tumours  M:F ratio is 3:2  Peak incidence: males 10-14 y.o.

think largest to smallest)  usually diaphyseal ◦ Flat bones      Pelvis (ilium) Ribs Vertebrae Scapula Clavicle . tibia. fibula . humerus.Location  Found most commonly in: ◦ Long bones (femur.

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Very uncommon .Associations or Risk Factors  EWS “Associations” -.skeletal abnormalities including endochrondroma and aneurysmal bone cyst - GU abnormalities including hypospadias and duplication of the renal collecting system Down syndrome Hereditary retinoblastoma - .

Clinical Presentation Pain and swelling about the affected limb with mild or moderate erythema  Pain . increasing Pathologic fracture in 10-15%  Systemic symptoms: fever or loss of energy seen in more advanced cases  .first sign in 50%  ◦ Characteristically worse at night.

Clinical Presentation May be found with incidental trauma  Loss of joint motion in patients with juxtaarticular lesions  Tumour in pelvis may present with gait abnormalities due to root compression. bowel or bladder dysfunction. or back pain  .

Clinical Presentation Mean duration of symptoms 9 months  20-25% present with metastatic disease  ◦ Lungs (38%) ◦ Bone (31%) ◦ Bone Marrow (11%) .

liver/kidney function tests.Diagnostic   History and physical examination Laboratory tests: ◦ CBC. ESR ◦ Urinalysis  Pathology ◦ Bone marrow aspirate and biopsy ◦ Biopsy (open preferred) . LDH.

 Radiologic tests ◦ ◦ ◦ ◦ ◦ Plain films of primary site CT/MRI of primary site CXR/CT of chest Whole body bone scan PET scan (in future)  Pre-therapy evaluation also includes echocardiogram/EKG .

Radiographic Presentation Metadiaphyseal or diaphyseal lesion in long bones  Lytic or sclerotic lesion. poorly marginated  Cortex varies from unaffected to thin and destroyed  .

Radiographic Presentation “onion skin” or Codman’s Triangle periosteal reaction  Periosteal and endosteal bone formation mimics osteosarcoma  Soft tissue mass is often seen  .

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Additional Investigations  Blood work: elevated LDH and alkaline phosphatase ◦ CBC (anemia. leukocytosis) Tc bone scan: screen for multifocal disease  Chest CT: presence of pulmonary metastases  .

Additional Investigations  MRI used to determine extent of soft tissue mass and intramedullary involvement ◦ Skip lesions with MRI of entire bone ◦ Relationship of tumor to adjacent neurovascular structures ◦ Assess response to chemo and radiation therapy .

Treatment Multidisciplinary approach must provide both local control and systemic therapy  Local control measures should not compromise systemic therapy  ◦ When treatment fails. it is usually due to the development of distant metastatic disease .

Treatment: Multimodal  Surgery ◦ local control where possible  Radiation ◦ local control where surgery not possible or incomplete  Chemotherapy ◦ control of micrometastases .

clavicle)  Bone defect able to be reconstructed with modest loss of function  May consider amputation if considerable growth remaining  Trend toward improved outcomes with chemo + surgery vs.Surgical Indications Expendable bone (fibula. XRT  . rib.

Radiation therapy Indications      Unresectable without significant morbidity Pelvic lesions Spine lesions Lung metastases May consider chemo + XRT to allow for surgical resection or add XRT if surgical margins positive .

adriamycin. melphalan Effective chemotherapy has improved local control rates achieved with radiation to 8590%  Role of SCT for high risk Ewing sarcoma still under investigation  . ifosfamide.Treatment: Chemotherapy All patients require chemotherapy  Active agents include  ◦ Vincristine. topotecan. cyclophosphamide. etoposide. dactinomycin.

Ewing’s sarcoma is a systemic disease with great majority having micrometastatic disease at presentation Evidenced by nearly uniformly fatal outcome in patients treated by irradiation or amputation of the primary tumor alone  .Prognosis  Like osteosarcoma.

Prognostic factors  Extent of disease ◦ Metastatic disease unfavorable ◦ Size of disease ???  Primary site ◦ Pelvis least favorable ◦ Distal bones and ribs most favorable  Age ◦ Younger (<10) more favorable  Histologic ??? ◦ Response to chemotherapy ◦ Neural differentiation .

Positive Prognostic Factors  Good radiologic response to induction chemotherapy Good pathologic response to induction chemotherapy  .

Negative Prognostic Factors Metastases at diagnosis  Over 8 cm in longest diameter  Large tumor volume  Pelvic location  High LDH levels  Over 17 years of age (controversial)  .

THANKS .

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