BACA EWING’S SARCOME

7/29/2019 BACA EWINGS SARCOME

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EWINGSSARCOME

TIM IV

IH,EG,DD,RM

James Ewing, 1921

Dr. James Ewing (1866 1943)


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Pediatric Bone Tumors

Benign

Osteochondroma

Osteoid Osteoma

Enchondroma

Chondroblastoma

Non-ossifying fibromaakabenign cortical defect

Hemangioma Eosinophilic

granuloma

Osteomyelitis

Malignant

Osteosarcoma

Ewing sarcoma

Malignant fibroushistiocytoma

Non-HodgkinLymphoma

Eosinophilic

granuloma


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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 56%

Ewing sarcoma 34%


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Ewing Sarcoma (EWS)

Represents a family of tumorsincluding

Ewing sarcoma of bone

extraosseous Ewing sarcoma and

peripheral neuroectodermal tumor (PNET)

of bone or soft tissue

2nd

most common bone tumor inchildren


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Epidemiology

1% of all childhood tumours

M:F ratio is 3:2

Peak incidence: males 10-14 y.o.,females 5-9 y.o.

Rare in African-Americans and Chinese


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Location

Found most commonly in:

Long bones (femur, tibia, humerus, fibula - think

largest to smallest)

usually diaphyseal

Flat bones

Pelvis (ilium)

Ribs

Vertebrae

Scapula

Clavicle


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Associations or Risk Factors EWS

Associations -- Very uncommon- skeletal abnormalities including

endochrondroma and aneurysmal bonecyst

- GU abnormalities including hypospadiasand duplication of the renal collectingsystem

- Down syndrome

- Hereditary retinoblastoma


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Clinical Presentation

Pain and swelling about the affected limb

with mild or moderate erythema

Pain - first sign in 50% Characteristically worse at night, increasing

Pathologic fracture in 10-15%

Systemic symptoms: fever or loss ofenergy seen in more advanced cases


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May be found with incidental trauma

Loss of joint motion in patients with juxta-

articular lesions Tumour in pelvis may present with gait

abnormalities due to root compression,

bowel or bladder dysfunction, or backpain


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Mean duration of symptoms 9 months 20-25% present with metastatic disease

Lungs (38%)

Bone (31%)

Bone Marrow (11%)


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Diagnostic

History and physical

examination

Laboratory tests:

CBC, liver/kidney

function tests, LDH, ESR

Urinalysis

Pathology

Bone marrow aspirate

and biopsy Biopsy (open preferred)


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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

includesechocardiogram/EKG


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Radiographic Presentation

Metadiaphyseal or

diaphyseal lesion in long

bones

Lytic or sclerotic lesion,

poorly marginated

Cortex varies fromunaffected to thin and

destroyed


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Radiographic Presentation

onion skin or Codmans

Triangle periosteal reaction

Periosteal and endosteal

bone formation mimics

osteosarcoma

Soft tissue mass is oftenseen


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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 pulmonarymetastases


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Additional Investigations

MRI used to determine extent of softtissue mass and intramedullary

involvement

Skip lesions with MRI of entire bone Relationship of tumor to adjacent

neurovascular structures

Assess response to chemo and radiationtherapy


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Treatment

Multidisciplinary approach mustprovide both local control and

systemic therapy

Local control measures should notcompromise systemic therapy

When treatment fails, it is usually due to

the development of distant metastaticdisease


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Treatment: Multimodal

Surgery local control where possible

Radiation local control where surgery not possible

or incomplete

Chemotherapy control of micrometastases


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Surgical Indications

Expendable bone (fibula, rib, clavicle)

Bone defect able to be reconstructed

with modest loss of function

May consider amputation ifconsiderable growth remaining

Trend toward improved outcomes with

chemo + surgery vs. XRT


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Radiation therapy

Indications Unresectable without significant morbidity

Pelvic lesions

Spine lesions

Lung metastases

May consider chemo + XRT to allow forsurgical resection or add XRT if surgicalmargins positive


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Treatment: Chemotherapy

All patients require chemotherapy

Active agents include

Vincristine, cyclophosphamide, adriamycin,

dactinomycin,

ifosfamide, etoposide, topotecan, melphalan

Effective chemotherapy has improved local

control rates achieved with radiation to 85-

90% Role of SCT for high risk Ewing sarcoma

still under investigation


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Prognosis

Like osteosarcoma, Ewings sarcomais a systemic disease with great

majority having micrometastatic

disease at presentation

Evidenced by nearly uniformly fatal

outcome in patients treated byirradiation or amputation of the

primary tumor alone


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Prognostic factors

Extent of disease Metastatic disease unfavorable

Size of disease ???

Primary site Pelvis least favorable

Distal bones and ribs most favorable

Age Younger (


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Positive Prognostic Factors

Good radiologic response to inductionchemotherapy

Good pathologic response to inductionchemotherapy


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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)


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THANKS


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