Ewing Sarcoma


Introduction:
Bone tumors are sixth most common group of cancers in children. Two common types of primary bone cancers found in children are osteosarcoma & Ewing sarcoma. Ewing sarcoma is the second most common tumor of the bone. It most often affects bones of the pelvis, the tibia, fibula, and femur, and can also begin in the soft tissues. This disease most often occurs in second sdecade of life that is between the ages of 10 and 20.They are small round cell undifferentiated tumors.


Types:
These are the Ewing Family of tumors which include Ewing sarcoma and PNET (Primitive neuroectodermal Tumors.

Symptoms:
1. Pain at the site of tumor
2. Swelling at the site of tumor
3. Weight loss
4. Anorexia
5. fever, malaise
6. cough

Prognostic factors:
1. Site of the tumor
2. The presence of metastasis
3. Size of the tumor
4. Age
5. Erythrocyte Sedimentation rate
6. Serum LDH levels

Investigations:
1. Plain X-Ray of the local site
2. CT scan or MRI of the primary tumor
3. Chest X-Ray
4. CT of the chest
5. Bone scan
6. Bone marrow aspiration and bone marrow biopsy

Treatment:
Ewing sarcoma is treated with multimodality treatment.

1. Surgery:
The options are

Amputation
Rotationplasty
Limb salvage surgery

Surgery is not effective for treating metastatic disease.

2. Chemotherapy:
Chemotherapy uses drugs like Ifosfamide, Etoposide, Adriamycin, Vincristine, Cyclophosphamide.It may be used before surgery to shrink a tumor so that it can be removed surgically, and will be used after surgery for any remaining cancer cells.

3. Radiation therapy:
Ewing sarcoma is very sensitive to radiation.When surgery is not possible because of large tumor mass, metastatic disease, certain site of cancer then radiation therapy is an effective answer.

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