ETIOLOGY SYMPTOMS C/F TREATMENT

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
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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.
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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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