Extraskeletal Ewing sarcoma (EES), is a cancer of soft tissue, a type of Ewing sarcoma that does not arise from bone.[1]
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]
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]
References
^ abcdWHO Classification of Tumours Editorial Board, ed. (2020). "2. Undifferentiated small round cell sarcoma of bone and soft tissue: Ewing sarcoma". Soft Tissue and Bone Tumours: WHO Classification of Tumours. Vol. 3 (5th ed.). Lyon (France): International Agency for Research on Cancer. pp. 323–325. ISBN978-92-832-4503-2. Archived from the original on 2021-06-13. Retrieved 2021-05-09.
