Ewing sarcoma is a highly malignant bone tumor that most commonly affects children and young adults. It is characterized by small, round cancer cells of unknown origin that invade bone and sometimes spread to soft tissues or other bones. Diagnosis involves imaging tests and biopsy showing the characteristic cells. Treatment typically involves chemotherapy, surgery to remove the tumor if possible, and sometimes radiation therapy. While Ewing sarcoma has a poor prognosis if untreated, multidisciplinary treatment with chemotherapy, surgery, and radiation can result in 5-year survival rates of 60-75% for patients without metastasis at diagnosis.

1. EWING SARCOMA
Dr.K.PRASHANTH
Pg in Orthopaedics
S V S Medical College
Mahaboobnagar
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2. HISTORY
James Stephen Ewing
American Pathologist (1866-1943)
Suffered from OM at the age of
14yrs. confined to bed for 2 yrs.
Served as Prof of Pathology for 33
yrs at Cornell Univ. New York.
Died of bladder cancer at 76yrs.
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3. Highly malignant bone tumor
PRECISE HISTOGENESIS IS UNKNOWN
• Round, small cell malignancy originating from bone marrow
cells
• Some believe that ES is a neurally derived small round cell
malignancy very similar to the so-called
Primitive Neuro Ectodermal Tumor (PNET).
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4. INCIDENCE
• 6th most common malignant tumour
• 2nd most common bone tumor in children
• 11% - 12% of all malignant tumours of bone
• Rare in blacks
4

5. TYPES
• Extraskeletal (soft
tissue ) ES
• Periosteal
• Atypical (large cell)
- cells are more
pleomorphic
• Multifocal
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All are characterised by recurrent
chromosomal translocation involving
11 & 22 (85%) & 21 &22 (15%)

6. LOCATION
LONG BONES: 50-60%
femur: 25%
tibia: 11%
humerus: 10%
FLAT BONES: 40%
pelvis: 14%
scapula
ribs: 6%
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7. LOCATION
• UPPER LIMB: 13%
• LOWER LIMB: 45%
femur most common
• PELVIS: 20%
• SPINE AND RIBS: 13%
sacrococcygeal most common
• SKULL / FACE: 2%
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8. LOCATION IN LONG BONES
almost always
metaphyseal or
diaphyseal
• metadiaphysis: 44%
• mid-diaphysis: 33%
• metaphysis: 15%
• epiphysis: 1-2%
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9. CLINICAL FEATURES
most common 2nd decade
• 5 - 25yrs. (90% below 20)
• Highest frequency 5-15yrs
• Mean age - 13yrs.
• Male predominance – 3:2
• Localised, painful, tender mass
• Systemic symp. – fever, malaise, weight loss
- mistaken for OM
- dissemination of tumor
• May metastasize to other bones
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10. DIAGNOSIS
• ESR is usually elevated
• Alkaline Phosphatase - normal (OS - elevated)
• Plain X Ray
• CT
• MRI
• Bone Scan
• PET Scan
• Biopsy - Wide Bore Needle Aspiration
- Open (incisional) biopsy
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11. PLAIN RADIOGRAPH
• The lesion is poorly defined
• Permeative or moth-eaten
type of bone destruction
• Aggressive periosteal
response
onion peel
codman triangle
sunburst appearance
• Large soft-tissue mass
• Occasionally, the bone lesion
itself is almost imperceptible,
with the soft-tissue mass being
the only prominent feature
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12. ONION PEEL PERIOSTEAL REACTION
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13. PLAIN RADIOGRAPHY
• A 24-year-old man
• pain and swelling - 8 wks;
fever
• destructive lesion of the distal
fibula - permeative type of bone
destruction
• lamellated periosteal reaction
• soft-tissue mass
• mimics infection
• biopsy revealed Ewing sarcoma.
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14. PLAIN RADIOGRAPHY
• 17-year-old boy
• significant degree of
sclerosis
• originally interpreted as
osteosarcoma
• biopsy revealed Ewing
sarcoma
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15. PLAIN RADIOGRAPHY
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16. SOFT TISSUE MASS - SCALLOPING
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17. CT SCAN
• reveals pattern of bone destruction
• provides information about the medullary
extension
• delineates extraosseous involvement
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18. CT SCAN
• 12-year-old boy
• poorly defined – permeative lesion
• aggressive periosteal reaction
• Axial CT - a large soft-tissue mass,
not clear on conventional study.
• complete obliteration of the marrow
cavity by tumor.
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19. CT SCAN
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20. MRI
• Demonstrates the extent of intraosseous and extraosseous
involvement of tumor (staging of tumor can be done)
• Effectively reveals extension through the epiphy. plate.
• T1-weighted images - intermediate to low signal intensity,
which becomes bright on T2 weighting.
• Hypocellular areas & areas of necrosis - lesser intensity
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21. MRI
• Contrast study by gadolinium- (Gd-DTPA) reveals signal
enhancement of the tumor on T1-weighted sequences
• Enhancement occurs only in the cellular areas, allowing
differentiation of the tumor from the peritumoral edema
• Evaluates response to chemotherapy & radiation treatment
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22. MRI
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23. MRI
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7 yr old girl, permeative & destructive lesion with
soft tissue shadow in metadiaphyseal region
MRI shows intra & extraosseous extension

24. RADIOISOTOPE BONE SCAN
• Technetium-99m methylene diphosphonate (99mTc-
MDP) - Increased uptake in areas of bone destruction
• Gallium-67-citrate - Soft tissue extension
• Scintigraphy – nonspecific but reliable in
identifying metastatic lesions
• Metastases may be present in up to 30% of cases
at time of diagnosis (mostly to bones & lungs)
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25. RADIOISOTOPE BONE SCAN
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26. RADIOISOTOPE BONE SCAN
• 13yr old girl
• ES of lt.iliac bone
• Due to morbidity with
surgical Rx, Radio &
Chemotherapy was
given
• Free of lesion 3.5yrs
after treatment
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27. DIFFERENTIAL DIAGNOSIS
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28. DIFFERENTIAL DIAGNOSIS
 OSTEOMYELITIS
Shorter duration of pain and less aggressive periosteal
reaction than Ewing’s
 EOSINOPHILIC GRANULOMA
Benign bone lesion with solid periosteal reaction
 OSTEOSARCOMA
Commonly occurs in long bones of young patients
Homogeneous, cloudlike osteoid deposition in soft tissues
 LYMPHOMA
Older age range
Clinically healthy
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29. GROSS FEATURES
• Gray - White
• Moist & glistening
• May be almost
liquid and may
resemble pus
(mistaken for
Osteomyelitis)
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30. HISTOPATHOLOGY
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Small round cell tumor with little intercellular stroma
Between the areas of highly cellular tumor, fibrous strands
compartmentalise the tumor
Low power

31. HISTOPATHOLOGY
• Cells are uniform and round
to oval
• Cytoplasm is scanty & lacy
• Nuclei are round to oval,
have a delicate ,finely
dispersed chromatin
• Nucleoli are inconspicuous
• Mitotic figures rare
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HIGH POWER

32. HISTOPATHOLOGY
Reticulin is poor
No evidence of Matrix
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IHC. Lymphoid markers CD99
strong positivity in short bones of
hand
Glycogen identifyable in
cytoplasm - PAS Stain

33. TREATMENT
MULTIDISCIPLINARY APPROACH
1. CHEMOTHERAPY - control of micrometastasis
2. SURGERY - local control where possible
3. RADIOTHERAPY- local control where surgery
is not possible or incomplete
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34. TREATMENT
• Systemic chemotherapy is the mainstay of treatment
with surgery and/or radiotherapy playing a role
depending of the location and size of the tumour
• usually treated with a preoperative course of
chemotherapy, either alone or combined with
radiation therapy, to shrink the tumor, followed by
wide resection
• the affected limb can be reconstructed with an
endoprosthesis or an allograft.
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35. TREATMENT
CHEMOTHERAPY
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36. CHEMOTHERAPY
Multiagent therapy for atleast 12 – 24 wks
prior to Local treatment
First line of Drugs
- Vincristine
- Adriamycin
- Cyclophosphamide
- Ifosphamide
- Itoposide
- Doxorubisine
Combination Therapy - VAC/IE, VAI, VADE
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37. CHEMOTHERAPY
SECOND LINE OF DRUGS (in relapsed & refractory cases)
- cyclophosphamide & topotecan
- temozolomide & irinotecan
- ifosfamide & etoposide
- ifosfamide, etoposide & corboplatin
- docetaxel & gemcitabin
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38. 38

39. SURGERY
• Chemo – cytoreduction makes resection possible
• Development of innovative surgical techniques:
Limb preservation & structural bone function preservation
• Local failure rates with RT – 9% to 25%
• INDICATIONS:
Expendable bones (Clavicle, fibula,rib)
Bone defect able to be reconstructed with modest loss of
function
Amputation if considerable growth remaining after RT
Limb salvage is preferred
curative surgery requires – wide local excision and
negative margin of at least 1 – 2cm
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40. • 11-year-old boy - typical appearance
of Ewing sarcoma
• poorly defined destructive lesion
• aggressive periosteal reaction and a
large soft-tissue mass
• Tomographic cut gives a better
picture of the periosteal response
and soft-tissue mass.
• 4-month course of chemotherapy
• lesion has become sclerotic
• periosteal reaction disappeared,
• soft-tissue mass has shrunk
• clavicle was then removed en bloc.
40

41. 41

42. RADIOTHERAPY
PRINCIPLES
- to sterilise tumor compatment before surgery
- potentially reduce the risk of dissemination during surgery
• It is a Radioresponsive Tumor
• RT in combination with Chemotherapy achieve local control but
complete surgery when feasible is the first choice of local control
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43. RADIOTHERAPY
Indications for RT after induction Chemotherapy
- Tumors where resection is not feasible
- For skull, face, vertebrae & pelvis
- where only intralesional resection is achievable
- Pt. with poor surgical risk
- Pt. refusing surgery
Palliative Radiotherapy
- Rib primary with pleural effision(RT for hemithorax)
- Lung metastasis (whoe lung RT)
- Pain palliation in advanced cases
- Isolated bone secondaries
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44. PROGNOSIS
• Used to be most lethal (5yr survival rate 15%) before the
advent of adjuvant chemotherapy
• During that era most patients were treated with radiotherapy
alone
• Surgical resection combined with chemotherapy produces
improved survival rates
• 60 – 75% Five Year Survival Rate without metastasis
• Cases with metastasis have less chance of being cured, but
should be treated aggressively as some will survive
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45. PROGNOSIS
Poor prognostic factors:
• Age >17ys
• Large size tumor > 8cm
• Metastases
• Pelvic & Sacral tumors
• Fever, Leucocytosis & Raised ESR at presentation
• <90% necrosis after Chemo
• Extraosseous soft tissue extension
• Postradiation medullary tumor
• Small bones(strong CD99+ve) – better prognosis
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46. RELAPSE
30 – 40% of Pts. develop relapse with <20% survival
• Early relapse - <2yrs.
Consider changing Chemotherapy
• Late relapse - >2yrs.
Continue previously used Chemotherapy
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47. CONCLUSION
• 2nd most common childhood malignant bone tumor
• Small round cell tumor with CD99 & PAS stain positive
• Painful with soft tissue mass
• Permeative with Lytic lesion & onion peel appearance
• Mimics infection clinically, radiologically & histologically
• Multimodal treatment approach
• Radiosensitive tumor
• Surgery where ever feasible with adjuvant
chemotherapy ensures promising 5yr survival rates
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48. THANK YOU …
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