Extraskeletal Ewing sarcoma
Medical condition
From Wikipedia, the free encyclopedia
Extraskeletal Ewing sarcoma (EES), is a cancer of soft tissue, a type of Ewing sarcoma that does not arise from bone.[1]
| 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] |
Signs and symptoms
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
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]
Treatment
Chemotherapy and surgical removal are options if the tumor is localised.[2] If it cannot be operated upon, radiation therapy may be effective.[2]
Epidemiology
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]
History
The condition was first reported by Melvin Tefft in 1969.[3]