Ewing sarcoma | |
---|---|
Other names | |
Micrograph of metastatic Ewing sarcoma (right of image) in normal lung (left of image). PAS stain. | |
Pronunciation | |
Specialty | Oncology |
Symptoms | Swell and pain near the tumor[2] |
Complications | Pleural effusion, paraplegia[3] |
Usual onset | 10 to 20 years old[4][3] |
Causes | Unknown[3] |
Diagnostic method | Tissue biopsy[2] |
Differential diagnosis | Osteosarcoma, neuroblastoma, osteomyelitis, eosinophilic granuloma[3] |
Treatment | Chemotherapy, radiation therapy, surgery, stem cell transplant[2] |
Prognosis | Five-year survival ~ 70%[4] |
Frequency | 1 per 1 million people (US)[4] |
Ewing sarcoma is a type of pediatric cancer[5] that forms in bone or soft tissue.[1] Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture.[2] The most common areas where it begins are the legs, pelvis, and chest wall.[4] In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis.[4] Complications may include a pleural effusion or paraplegia.[3]
It is a type of small round cell sarcoma.[1] The cause of Ewing sarcoma is unknown, most cases appearing to occur randomly.[3] Though not strongly associated with known hereditary cancer syndromes, accumulating evidence suggests a strong inherited risk factor,[6] identifying a genetic component having multiple chromosome loci associated with Ewing sarcoma susceptibility.[5] Sometimes Ewing sarcoma is associated with a germline mutation.[1] The underlying mechanism often involves a genetic change known as a reciprocal translocation.[4] Diagnosis is based on biopsy of the tumor.[2]
Treatment often includes chemotherapy, radiation therapy, surgery, and stem cell transplant.[7][2] Targeted therapy and immunotherapy are being studied.[2] Five-year survival is about 70%.[4] A number of factors, however, affect this estimate.[4]
In 1920, James Ewing discerned that these tumors are a distinct type of cancer.[8][9] It affects approximately one in a million people per year in the United States.[4] Ewing sarcoma occurs most often in teenagers and young adults and represents 2% of childhood cancers.[2][3] Caucasians are affected more often than African Americans or Asians, while males are affected more often than females.[10][4]