Ewing sarcoma

Ewing's sarcoma is the 3rd most common primary malignant bone tumor in the world. It affects people at first 2 decades. In this presentation, every important aspect of this bone tumor has been described extensively but in brief.

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

2. Introduction
•It is the 3
rd
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: Diaphysisof long bone.
•Common bones involved: (in decreasing
order)
1.Femur,
2.Tibia,
3.Fibula,
4.Humerus,
5.Flat bones.

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

5. Pathology
•Lobulatedlarge mass over diaphysisof long
bone.
•When hemorrhage occurs, it appears like a red
current jelly.
•Extensive medularyinvasion and destruction
of endosteumand cortex.
•Prominent peiostealnew bone formation.
•Sometimes, the tumor is liquefied so as to
resemble a pus.

6. Histology
•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:
Diaphyseallesion with irregular destruction (Moth
eaten appearance).
Periostealnew 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 intraosseusand
extraosseusextension 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 hematogenousand
lymphatogenousroutes.
•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.Debulkingsurgery.
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/
ActinomycinD/ Doxorubicin/ Vincristineetc.

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

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