User:Whispyhistory/soft tissue and bone tumors/undifferentiated small round cell sarcomas of bone and soft tissue

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Undifferentiated small round cell sarcomas of bone and soft tissue.[1]

  • Ewing sarcoma
  • Distinct and sometimes termed related entities
    • Round cell sarcoma with EWSR1- non-ETS fusions
    • CIC-rearranged sarcoma
    • Sarcoma with BCOR genetic alterations


Extraskeletal Ewing sarcoma (EES), is a cancer of soft tissue, a type of Ewing sarcoma that does not arise from bone.[2] Typical symptoms include a rapidly growing lump and pain at the site of the tumor.[3] It can occur in a wide range of parts of the body.[2] It grows rapidly, with the upper leg, upper arms, bottom and shoulders being the most common sites to be affected.[3] At presentation, a quarter of cases have already spread; typically to lungs, bone and bone marrow.[3]

It belongs to the Ewing family of tumors.[4] 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.[3] Fluorodeoxyglucose-positron emission tomography is more accurate than a bone scan in detecting spread, and can be used to monitor response to treatment.[3] Chemotherapy and surgical removal are options if the tumor is localised.[3] If it cannot be operated upon, radiation therapy may be effective.[3] Five-year survival with treatment is over 80%.[5]

The tumor is rare.[3] It accounts for around 12% of cases of Ewing sarcoma.[2] 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 5-year olds and over 35-year olds.[3] There does not appear to be any association with ethnicity or gender.[3] The condition was first reported by Melvin Tefft in 1969.[6]


  1. Davis, Jessica L.; Rudzinski, Erin R. (1 September 2020). "Small Round Blue Cell Sarcoma Other Than Ewing Sarcoma: What Should an Oncologist Know?". Current Treatment Options in Oncology. 21 (11): 90. doi:10.1007/s11864-020-00785-1. ISSN 1534-6277. PMID 32875423.
  2. 2.0 2.1 2.2 WHO 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. ISBN 978-92-832-4503-2. Archived from the original on 2021-06-13. Retrieved 2021-05-09.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 3.9 Abboud, A; Masrouha, K; Saliba, M; Haidar, R; Saab, R; Khoury, N; Tawil, A; Saghieh, S (May 2021). "Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis". Oncology letters. 21 (5): 354. doi:10.3892/ol.2021.12615. PMID 33747211.
  4. "Extraskeletal Ewing sarcoma". 2 February 2011. Archived from the original on 22 January 2022. Retrieved 22 June 2022.
  5. Wright, Alexandra; Desai, Madhura; Bolan, Candice W.; Badawy, Mohamed; Guccione, Jeffrey; Rao Korivi, Brinda; Pickhardt, Perry J.; Mellnick, Vincent M.; Lubner, Meghan G.; Chen, Longwen; Elsayes, Khaled M. (27 May 2022). "Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review". Radiographics: A Review Publication of the Radiological Society of North America, Inc: 210226. doi:10.1148/rg.210226. ISSN 1527-1323. PMID 35622491. Archived from the original on 27 May 2022. Retrieved 22 June 2022.
  6. Veselis, Clinton A.; Awan, Omer; Thomas, Ashanth; Ling, Stephen; Jonnalagadda, Padmaja; Aneja, Amandeep; Ali, Sayed (May 2021). "Bone Tumors Occurring in the Soft Tissues: A Review of the Clinical, Imaging, and Histopathologic Findings". Current Problems in Diagnostic Radiology. 50 (3): 419–429. doi:10.1067/j.cpradiol.2020.06.004. ISSN 1535-6302. PMID 32665061. Archived from the original on 2021-03-30. Retrieved 2022-06-20.