| Extraskeletal Ewing sarcoma | |
|---|---|
| Other names | Extraosseous Ewing sarcoma |
| Specialty | Oncology [1] |
| Symptoms | Pain at the site of the tumor [2] |
| Complications | Spread [2] |
| Usual onset | Rapid, <5years and >35years of age [2] |
| Diagnostic method | Medical imaging [2] |
| Treatment | Chemotherapy, surgical removal, radiation therapy [2] |
| Frequency | 0.4 per million, males=females [2] |
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. [2] 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]