Extraskeletal Ewing sarcoma
| 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]
Signs and symptoms
[edit]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
[edit]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
[edit]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
[edit]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
[edit]The condition was first reported by Melvin Tefft in 1969.[3]
References
[edit]- ^ a b c d 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.
- ^ a b c d e f g h i j k l m n o p q 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. PMC 7967932. PMID 33747211.
- ^ 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. S2CID 220530415. Archived from the original on 2021-03-30. Retrieved 2022-06-20.
Further reading
[edit]- Tefft, M.; Vawter, G. F.; Mitus, A. (June 1969). "Paravertebral "round cell" tumors in children". Radiology. 92 (7): 1501–1509. doi:10.1148/92.7.1501. ISSN 0033-8419. PMID 5799839.