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.
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• It is the 3rd most common primary malignant
• Arises from endothelial cells of bone marrow.
• Age: First 2 decades of life.
• Common in males.
• Uncommon in blacks.
• Location affected: Diaphysis of long bone.
• Common bones involved: (in decreasing
5. Flat bones.
Throbbing, intermittent pain.
Pain worst at night.
Red and warm skin.
Swelling over diaphyseal region.
Generalized illness and pyrexia.
Mimics acute osteomyelitis.
• Lobulated large mass over diaphysis of long
• When hemorrhage occurs, it appears like a red
• 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.
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).
• CT Scan:
Detect the extent of cortical destruction.
CT scan chest: Detection of metastasis.
Detection of the degree of intraosseus and
extraosseus extension of tumor.
Initial staging and surgical planning.
Assessment of response of the tumor to
• Bone scan:
Detection of the extent of tumour
Detection of the skip lesions in the same
Detection of distant metastasis.
• Repeated episodes of exacerbation and
remission is characteristic.
• Metastasize by hematogenous and
• It obeys the course of bone to bone
metastasis (another one example is
• Common bones where Ewing’s sarcoma
metastasizes are skull, vertebra, rib and lung.
• Ewing’s sarcoma is highly radiosensitive
• 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
• Surgery has a very little role in the
management of Ewing sarcoma.
• The available options are:
1. Debulking surgery.
2. Limb preservation surgery.
• It is the mainstay of treatment of Ewing’s
• Response to chemotherapy is the most
important prognostic factor in this disease.
• Common agents used are: Ifosfamide/
Actinomycin D/ Doxorubicin/ Vincristine etc.
Best result can be achieved only by
combining all the 3 options available.
Site: Humerus and pelvis-> Bad prognosis.
Stage: Metastasis-> Bad prognosis.
Response to chemotherapy.
High LDH level.
c-MIC/ ki-67 gene expression.