| Extraskeletal Ewing sarcoma | |
| Synonyms: | Extraosseous Ewing sarcoma |
| Field: | Oncology |
| Symptoms: | Pain at the site of the tumor |
| Complications: | Spread |
| Onset: | Rapid, <5years and >35years of age |
| Diagnosis: | Medical imaging |
| Treatment: | Chemotherapy, surgical removal, radiation therapy |
| Frequency: | 0.4 per million, males=females |
Extraskeletal Ewing sarcoma (EES), is a cancer of soft tissue, a type of Ewing sarcoma that does not arise from bone.[1]
It belongs to the Ewing family of tumors. Typical symptoms include pain at the site of the tumor.[2] It can occur in a wide range of parts of the body.[1] It grows rapidly, with the upper leg, upper arms, bottom and shoulders being the most common sites to be affected.[2] At presentation, a quarter of cases have already spread; typically to lungs, bone and bone marrow.[2]
Diagnosis is by medical imaging, with MRI being more accurate than CT scan, and confirmed by CT-guided or ultrasound-guided core-needle biopsy once a chest CT has excluded spread to lungs.[2] Fluorodeoxyglucose-positron emission tomography is more accurate than a bone scan in detecting spread, and can be used to monitor response to treatment.[2]
Chemotherapy and surgical removal are options if the tumor is localised.[2] If it cannot be operated upon, radiation therapy may be effective.[2]
The tumor is rare.[2] It accounts for around 12% of cases of Ewing sarcoma.[1] It is 10 times less common than Ewing sarcoma of bone and occurs in around 1.4 per million people, with a greater likelihood in under five-year olds and over 35-year olds.[2] There does not appear to be any association with ethnicity or gender.[2]
The condition was first reported by Melvin Tefft in 1969.[3]