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EWING SARCOMA
Dr.K.PRASHANTH
Pg in Orthopaedics
S V S Medical College
Mahaboobnagar
1
HISTORY
James Stephen Ewing
American Pathologist (1866-1943)
Suffered from OM at the age of
14yrs. confined to bed for 2 y...
Highly malignant bone tumor
PRECISE HISTOGENESIS IS UNKNOWN
• Round, small cell malignancy originating from bone marrow
ce...
INCIDENCE
• 6th most common malignant tumour
• 2nd most common bone tumor in children
• 11% - 12% of all malignant tumours...
TYPES
• Extraskeletal (soft
tissue ) ES
• Periosteal
• Atypical (large cell)
- cells are more
pleomorphic
• Multifocal
5
A...
LOCATION
LONG BONES: 50-60%
femur: 25%
tibia: 11%
humerus: 10%
FLAT BONES: 40%
pelvis: 14%
scapula
ribs: 6%
6
LOCATION
• UPPER LIMB: 13%
• LOWER LIMB: 45%
femur most common
• PELVIS: 20%
• SPINE AND RIBS: 13%
sacrococcygeal most com...
LOCATION IN LONG BONES
almost always
metaphyseal or
diaphyseal
• metadiaphysis: 44%
• mid-diaphysis: 33%
• metaphysis: 15%...
CLINICAL FEATURES
most common 2nd decade
• 5 - 25yrs. (90% below 20)
• Highest frequency 5-15yrs
• Mean age - 13yrs.
• Mal...
DIAGNOSIS
• ESR is usually elevated
• Alkaline Phosphatase - normal (OS - elevated)
• Plain X Ray
• CT
• MRI
• Bone Scan
•...
PLAIN RADIOGRAPH
• The lesion is poorly defined
• Permeative or moth-eaten
type of bone destruction
• Aggressive periostea...
ONION PEEL PERIOSTEAL REACTION
12
PLAIN RADIOGRAPHY
• A 24-year-old man
• pain and swelling - 8 wks;
fever
• destructive lesion of the distal
fibula - perme...
PLAIN RADIOGRAPHY
• 17-year-old boy
• significant degree of
sclerosis
• originally interpreted as
osteosarcoma
• biopsy re...
PLAIN RADIOGRAPHY
15
SOFT TISSUE MASS - SCALLOPING
16
CT SCAN
• reveals pattern of bone destruction
• provides information about the medullary
extension
• delineates extraosseo...
CT SCAN
• 12-year-old boy
• poorly defined – permeative lesion
• aggressive periosteal reaction
• Axial CT - a large soft-...
CT SCAN
19
MRI
• Demonstrates the extent of intraosseous and extraosseous
involvement of tumor (staging of tumor can be done)
• Effec...
MRI
• Contrast study by gadolinium- (Gd-DTPA) reveals signal
enhancement of the tumor on T1-weighted sequences
• Enhanceme...
MRI
22
MRI
23
7 yr old girl, permeative & destructive lesion with
soft tissue shadow in metadiaphyseal region
MRI shows intra & e...
RADIOISOTOPE BONE SCAN
• Technetium-99m methylene diphosphonate (99mTc-
MDP) - Increased uptake in areas of bone destructi...
RADIOISOTOPE BONE SCAN
25
RADIOISOTOPE BONE SCAN
• 13yr old girl
• ES of lt.iliac bone
• Due to morbidity with
surgical Rx, Radio &
Chemotherapy was...
DIFFERENTIAL DIAGNOSIS
27
DIFFERENTIAL DIAGNOSIS
 OSTEOMYELITIS
Shorter duration of pain and less aggressive periosteal
reaction than Ewing’s
 EOS...
GROSS FEATURES
• Gray - White
• Moist & glistening
• May be almost
liquid and may
resemble pus
(mistaken for
Osteomyelitis...
HISTOPATHOLOGY
30
Small round cell tumor with little intercellular stroma
Between the areas of highly cellular tumor, fibr...
HISTOPATHOLOGY
• Cells are uniform and round
to oval
• Cytoplasm is scanty & lacy
• Nuclei are round to oval,
have a delic...
HISTOPATHOLOGY
Reticulin is poor
No evidence of Matrix
32
IHC. Lymphoid markers CD99
strong positivity in short bones of
h...
TREATMENT
MULTIDISCIPLINARY APPROACH
1. CHEMOTHERAPY - control of micrometastasis
2. SURGERY - local control where possibl...
TREATMENT
• Systemic chemotherapy is the mainstay of treatment
with surgery and/or radiotherapy playing a role
depending o...
TREATMENT
CHEMOTHERAPY
35
CHEMOTHERAPY
Multiagent therapy for atleast 12 – 24 wks
prior to Local treatment
First line of Drugs
- Vincristine
- Adria...
CHEMOTHERAPY
SECOND LINE OF DRUGS (in relapsed & refractory cases)
- cyclophosphamide & topotecan
- temozolomide & irinote...
38
SURGERY
• Chemo – cytoreduction makes resection possible
• Development of innovative surgical techniques:
Limb preservatio...
• 11-year-old boy - typical appearance
of Ewing sarcoma
• poorly defined destructive lesion
• aggressive periosteal reacti...
41
RADIOTHERAPY
PRINCIPLES
- to sterilise tumor compatment before surgery
- potentially reduce the risk of dissemination duri...
RADIOTHERAPY
Indications for RT after induction Chemotherapy
- Tumors where resection is not feasible
- For skull, face, v...
PROGNOSIS
• Used to be most lethal (5yr survival rate 15%) before the
advent of adjuvant chemotherapy
• During that era mo...
PROGNOSIS
Poor prognostic factors:
• Age >17ys
• Large size tumor > 8cm
• Metastases
• Pelvic & Sacral tumors
• Fever, Leu...
RELAPSE
30 – 40% of Pts. develop relapse with <20% survival
• Early relapse - <2yrs.
Consider changing Chemotherapy
• Late...
CONCLUSION
• 2nd most common childhood malignant bone tumor
• Small round cell tumor with CD99 & PAS stain positive
• Pain...
THANK YOU …
48
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EWINGS SARCOMA

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EWINGS SARCOMA

  1. 1. EWING SARCOMA Dr.K.PRASHANTH Pg in Orthopaedics S V S Medical College Mahaboobnagar 1
  2. 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. 2
  3. 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). 3
  4. 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. 5. TYPES • Extraskeletal (soft tissue ) ES • Periosteal • Atypical (large cell) - cells are more pleomorphic • Multifocal 5 All are characterised by recurrent chromosomal translocation involving 11 & 22 (85%) & 21 &22 (15%)
  6. 6. LOCATION LONG BONES: 50-60% femur: 25% tibia: 11% humerus: 10% FLAT BONES: 40% pelvis: 14% scapula ribs: 6% 6
  7. 7. LOCATION • UPPER LIMB: 13% • LOWER LIMB: 45% femur most common • PELVIS: 20% • SPINE AND RIBS: 13% sacrococcygeal most common • SKULL / FACE: 2% 7
  8. 8. LOCATION IN LONG BONES almost always metaphyseal or diaphyseal • metadiaphysis: 44% • mid-diaphysis: 33% • metaphysis: 15% • epiphysis: 1-2% 8
  9. 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 9
  10. 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 10
  11. 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 11
  12. 12. ONION PEEL PERIOSTEAL REACTION 12
  13. 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. 13
  14. 14. PLAIN RADIOGRAPHY • 17-year-old boy • significant degree of sclerosis • originally interpreted as osteosarcoma • biopsy revealed Ewing sarcoma 14
  15. 15. PLAIN RADIOGRAPHY 15
  16. 16. SOFT TISSUE MASS - SCALLOPING 16
  17. 17. CT SCAN • reveals pattern of bone destruction • provides information about the medullary extension • delineates extraosseous involvement 17
  18. 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. 18
  19. 19. CT SCAN 19
  20. 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 20
  21. 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 21
  22. 22. MRI 22
  23. 23. MRI 23 7 yr old girl, permeative & destructive lesion with soft tissue shadow in metadiaphyseal region MRI shows intra & extraosseous extension
  24. 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) 24
  25. 25. RADIOISOTOPE BONE SCAN 25
  26. 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 26
  27. 27. DIFFERENTIAL DIAGNOSIS 27
  28. 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 28
  29. 29. GROSS FEATURES • Gray - White • Moist & glistening • May be almost liquid and may resemble pus (mistaken for Osteomyelitis) 29
  30. 30. HISTOPATHOLOGY 30 Small round cell tumor with little intercellular stroma Between the areas of highly cellular tumor, fibrous strands compartmentalise the tumor Low power
  31. 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 31 HIGH POWER
  32. 32. HISTOPATHOLOGY Reticulin is poor No evidence of Matrix 32 IHC. Lymphoid markers CD99 strong positivity in short bones of hand Glycogen identifyable in cytoplasm - PAS Stain
  33. 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 33
  34. 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. 34
  35. 35. TREATMENT CHEMOTHERAPY 35
  36. 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 36
  37. 37. CHEMOTHERAPY SECOND LINE OF DRUGS (in relapsed & refractory cases) - cyclophosphamide & topotecan - temozolomide & irinotecan - ifosfamide & etoposide - ifosfamide, etoposide & corboplatin - docetaxel & gemcitabin 37
  38. 38. 38
  39. 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 39
  40. 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. 41
  42. 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 42
  43. 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 43
  44. 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 44
  45. 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 45
  46. 46. RELAPSE 30 – 40% of Pts. develop relapse with <20% survival • Early relapse - <2yrs. Consider changing Chemotherapy • Late relapse - >2yrs. Continue previously used Chemotherapy 46
  47. 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 47
  48. 48. THANK YOU … 48

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