Extraskeletal Ewing sarcoma

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

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]

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]

References

^ ^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.

External links

[WHOblue2020-1] 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.

[Abboud2021-2] ^ ^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.

[Veselis2021-3] 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.

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