Ewing’s sarcoma is the common term given to group of bone cancers that develops in children and adolescents. It is also known by name ESFT (Ewing sarcoma family of tumors) and is a rare type of bone cancer. If treated in early stages Ewing sarcoma can be cured completely. This cancer begins in bone cells and hence is called as primary bone cancer. Some types of cancer originate in some other place and metastasize to bones which are called as secondary cancer. This cancer was first explained by James Ewing and hence the name Ewing sarcoma. It occurs in lengthy bones like arms and thigh bones but sometimes on the chest and pelvic bones also.
Though many theories are suggested for the cause of Ewing cancer, nothing has proof. The tumor is believed to originate from a primitive cell of embryologic tissue or from the neural crest. Another theory suggests that Ewing cancer is very much like to the PNET (primitive neuro ectodermal tumor). Sudden changes that causes gene mutation might be a cause of Ewing cancer which affects the gene by name EWS in chromosome 22.
Risk Factors :
Ewing sarcoma is a rare type of bone cancer and only 2-3 persons are affected in one million. In more than 90% of cases, Ewing sarcoma affects age group of 5-15 years. In rare cases, ES occurs after 25 years. Boys are more affected by ES than girls for reasons unknown. It is common in white people when compared with blacks.
The symptoms of Ewing sarcoma are often confusing with other bone injuries. Some of the common signs are swelling of bone, pain in the affected area (leg or arm), limited joint movement and broken bone without any apparent cause. The affected child will lose weight and feel extremely tired if the cancer has spread to other organs.
If you notice any lumps or lesions on the arms, legs or back of your children which does not go away quickly it can be an indication of Ewing cancer. In some cases if the bone cancer has affected the chest wall it would not show any symptoms until the cancer has widely spread. Hence in such cases it is difficult to diagnose Ewing sarcoma.
Your doctor will complete the physical exam and listen to the symptoms of the child. He would do a biopsy by removing a small tissue sample of the tumor from the affected bone. He would request for blood test to check if the levels of RBC and WBC are unusually high. He may order for imaging tests like X-ray, CT/MRI scanning to get a clear picture of the disease. Bone scanning is done by injecting a radioactive material into it and monitoring the results using special camera. In some cases PET scan is also done.
Like all other types of cancer, Ewing sarcoma has to be separated into stages. The stage of the cancer is absolutely necessary to find out how far the cancerous cells have spread into the body.
- In stage 1, the cancer is just a small tumor inside the bone.
- In stage 2, the cancerous cells have affected the nearby tissues also.
- In stage 3, the tumor has started affecting the entire bone of the affected area including the lymph nodes.
- In stage 4, the cancerous cells have metastasized to distant areas of the body like the lungs and the liver.
The survival rate and the method of treatment are decided based on the stage of cancer. About 70% of the cases with localized tumor (First stage) would survive for 5 years. If the cancer has spread to other organs then only 20-30% of the cases can survive for 5 years. However this varies definitely on each individual’s age and health condition. There are some children who would respond well to chemotherapy who can survive for more years than expected.
In the first two stages, Ewing sarcoma is treated with chemotherapy. Anticancer medications are given intravenously so that it destroys the tumor completely. It would help in shrinking the tumor so that surgery can be done easily. Surgery is the best option if the size of the tumor is big and is near vital organs and the cancerous cells have invaded the lymph nodes.
In many cases radiation therapy is done even after surgery for destroying the remaining cancer cells from the body. Chemo and radiation therapy is to be continued for many months until the doctor is satisfied that the cancer cells are totally destroyed. A combination of these therapies works out well in many cases. After the surgery imaging test will be done to check the status of cancer regularly which can be managed by radiation and chemotherapy. Many doctors from various fields of expertise like oncology, orthopedics, radiation specialist and musculoskeletal specialist will have to work together for getting the best outcome.
Follow Up :
There is every chance for the child to develop secondary cancers after some years and hence regular checkups are necessary to restore health after surgery.