Ewing's sarcoma is the third most common primary malignant bone tumor that arises from endothelial cells of bone marrow. It commonly affects males in their first two decades of life, presenting with pain in the diaphysis of long bones like the femur and tibia. Diagnostic tests include blood tests showing anemia and elevated markers, x-rays revealing moth-eaten bone destruction and onion peel periosteal reaction, and MRI or CT scans assessing tumor extent and staging. Treatment involves a combination of chemotherapy, surgery if possible for debulking or limb preservation, and radiotherapy, with chemotherapy being the mainstay. Prognosis depends on factors like tumor site, stage, size, and response to initial chemotherapy.

1. EWING’S SARCOMA
PRITHWIRAJ MAITI
FINAL YEAR MBBS
R.G.KAR MEDICAL COLLEGE
5.3.2014

2. Introduction
• It is the 3rd most common primary malignant
bone tumor.
• Arises from endothelial cells of bone marrow.
• Age: First 2 decades of life.
• Common in males.
• Uncommon in blacks.

3. • Location affected: Diaphysis of long bone.
• Common bones involved: (in decreasing
order)
1. Femur,
2. Tibia,
3. Fibula,
4. Humerus,
5. Flat bones.

4. Clinical features
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Throbbing, intermittent pain.
Pain worst at night.
Red and warm skin.
Swelling over diaphyseal region.
Generalized illness and pyrexia.
Mimics acute osteomyelitis.

5. Pathology
• Lobulated large mass over diaphysis of long
bone.
• When hemorrhage occurs, it appears like a red
current jelly.
• Extensive medulary invasion and destruction
of endosteum and cortex.
• Prominent peiosteal new bone formation.
• Sometimes, the tumor is liquefied so as to
resemble a pus.

6. Histology
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Highly cellular.
Little intercellular matrix.
Necrosis is common.
Cells are arranged around the vessels.
PAS +Ve (due to presence of glycogen).
HBA71 +Ve (it is an immunological marker).

8. Investigations
• Blood:
 Anemia,
 Leucocytosis,
 Raised ESR,
 Raised LDH.
• X-Ray:
 Diaphyseal lesion with irregular destruction (Moth
eaten appearance).
 Periosteal new bone formation (Onion peel
appearance).
 X-Ray chest (to detect metastasis).

10. • CT Scan:
 Detect the extent of cortical destruction.
 CT scan chest: Detection of metastasis.
• MRI:
 Detection of the degree of intraosseus and
extraosseus extension of tumor.
 Initial staging and surgical planning.
 Assessment of response of the tumor to
chemotherapeutic regimen.

11. • Bone scan:
Detection of the extent of tumour
involvement.
Detection of the skip lesions in the same
bone.
Detection of distant metastasis.

12. Course
• Repeated episodes of exacerbation and
remission is characteristic.
• Metastasize by hematogenous and
lymphatogenous routes.
• It obeys the course of bone to bone
metastasis (another one example is
osteosarcoma).
• Common bones where Ewing’s sarcoma
metastasizes are skull, vertebra, rib and lung.

13. Management of Ewing’s sarcoma
1. Radiotherapy,
2. Surgery,
3. Chemotherapy.

14. Radiotherapy
• Ewing’s sarcoma is highly radiosensitive
tumour.
• Radiotherapy has dramatic effect on Ewing’s
sarcoma (Melts like snow).
• But recurrence rates are high in case of
radiotherapy alone, survival rates are also not
satisfactory.

15. Surgery
• Surgery has a very little role in the
management of Ewing sarcoma.
• The available options are:
1. Debulking surgery.
2. Limb preservation surgery.

16. Systemic Chemotherapy
• It is the mainstay of treatment of Ewing’s
sarcoma.
• Response to chemotherapy is the most
important prognostic factor in this disease.
• Common agents used are: Ifosfamide/
Actinomycin D/ Doxorubicin/ Vincristine etc.

17. Comment
Best result can be achieved only by
combining all the 3 options available.

18. Prognostic Factors
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Site: Humerus and pelvis-> Bad prognosis.
Stage: Metastasis-> Bad prognosis.
Tumour size.
Response to chemotherapy.
Male gender.
High LDH level.
Anemia.
c-MIC/ ki-67 gene expression.