Bone Cancer-II

Ewings Sarcoma:

What is Ewings Sarcoma

Ewings sarcoma is a primary malignant "small round cell" tumour of bone. The small cells that make up the tumour resemble those of lymphoma or neuroblastoma, and current evidence suggests that the tumour cells are related to nerve cells (have a neural phenotype).

Who gets Ewings Sarcoma?

Ewings sarcoma constitutes approximately 10-15% of all bone sarcomas. It is the bone tumour with the youngest age predilection - being common in adolescence, and has a peak incidence in the second decade of life.

Predisposing Factors

Ewings sarcomas arise in teenage years when bone growth is most rapid. It is believed that the rapid bone growth predisposes to the development of the cancer. In approximately 85% of Ewings sarcomas there is a specific genetic cause.

Progression

The disease can be very aggressive, hence it is considered a systemic disease. Local growth is often accompanied by systemic metastasis (spreading) - to lung, other bones, and bone marrow.

Probable Outcomes

Ewings sarcoma is a curable disease, even with the presence of metastatic disease, especially in children under the age of 11. Patients with lesions in the distal arm (below the elbow) or distal leg (below the calf) have a 5-year survival rate of 80% with effective treatment.

How is Ewings Sarcoma Diagnosed?

Blood Tests: Serum alkaline phosphatase is usually raised, and hypercalcaemia may be present with widespread disease.

X-ray: The plain radiograph may show characteristic "onion peel" periosteal reaction with a generous soft tissue mass.

How is Ewings Sarcoma treated?

The treatment of Ewings sarcoma usually involves chemotherapy and surgery, with or without radiotherapy.

Systemic chemotherapy is often used before surgery - involving doxorubicin, cyclophosphamide, etoposide, vincristine, and dactinomycin.

Local treatment of the tumour (often after chemotherapy) involves surgical resection, usually with limb salvage or radiation therapy.

Analgesics and anti-inflammatory drugs are important for pain relief.

Ewings Sarcoma References

1. Braunwald, Fauci, Kasper, Hauser, Longo, Jameson. Harrison's Principles of Internal Medicine. 15th Edition. McGraw-Hill. 2001
   
2. Cotran RS, Kumar V, Collins T. Robbins Pathological Basis of Disease Sixth Ed. WB Saunders Company 1999.
   
3. Kumar P, Clark M. Clinical Medicine. Fourth Ed. WB Saunders, 1998.
   
   
   

   Regimens Used in the Treatment of This Disease:

   • Dacarbazine

   Drugs/Products Used in the Treatment of This Disease:

   • Doxorubicin Hydrochloride Injection (DBL)
     (Doxorubicin hydrochloride)
     
     
   • Doxorubicin Hydrochloride Injection USP
     (Doxorubicin hydrochloride)
     
     
   • Etoposide Injection
     (Etoposide)
     
     
   • Cycloblastin
     (Cyclophosphamide)
     
     
   • Holoxan
     (Ifosfamide)