Ewing sarcoma

Ewing sarcoma
Other names
  • Ewing's sarcoma
  • Peripheral primitive neuroectodermal tumor and Askin tumor (not recommended)[1]
  • Ewing sarcoma family of tumors[2]
Micrograph of metastatic Ewing sarcoma (right of image) in normal lung (left of image). PAS stain.
Pronunciation
SpecialtyOncology
SymptomsSwell and pain near the tumor[2]
ComplicationsPleural effusion, paraplegia[3]
Usual onset10 to 20 years old[4][3]
CausesUnknown[3]
Diagnostic methodTissue biopsy[2]
Differential diagnosisOsteosarcoma, neuroblastoma, osteomyelitis, eosinophilic granuloma[3]
TreatmentChemotherapy, radiation therapy, surgery, stem cell transplant[2]
PrognosisFive-year survival ~ 70%[4]
Frequency1 per 1 million people (US)[4]

Ewing sarcoma is a type of pediatric cancer[5] that forms in bone or soft tissue.[1] Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture.[2] The most common areas where it begins are the legs, pelvis, and chest wall.[4] In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis.[4] Complications may include a pleural effusion or paraplegia.[3]

It is a type of small round cell sarcoma.[1] The cause of Ewing sarcoma is unknown, most cases appearing to occur randomly.[3] Though not strongly associated with known hereditary cancer syndromes, accumulating evidence suggests a strong inherited risk factor,[6] identifying a genetic component having multiple chromosome loci associated with Ewing sarcoma susceptibility.[5] Sometimes Ewing sarcoma is associated with a germline mutation.[1] The underlying mechanism often involves a genetic change known as a reciprocal translocation.[4] Diagnosis is based on biopsy of the tumor.[2]

Treatment often includes chemotherapy, radiation therapy, surgery, and stem cell transplant.[7][2] Targeted therapy and immunotherapy are being studied.[2] Five-year survival is about 70%.[4] A number of factors, however, affect this estimate.[4]

In 1920, James Ewing discerned that these tumors are a distinct type of cancer.[8][9] It affects approximately one in a million people per year in the United States.[4] Ewing sarcoma occurs most often in teenagers and young adults and represents 2% of childhood cancers.[2][3] Caucasians are affected more often than African Americans or Asians, while males are affected more often than females.[10][4]

  1. ^ 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.
  2. ^ a b c d e f g h i "Ewing Sarcoma Treatment". National Cancer Institute. 25 January 2019. Retrieved 3 February 2019.
  3. ^ a b c d e f g "Ewing Sarcoma". NORD (National Organization for Rare Disorders). 2013. Retrieved 4 February 2019.
  4. ^ a b c d e f g h i j "Ewing Sarcoma Treatment". National Cancer Institute. 31 January 2019. Retrieved 4 February 2019.
  5. ^ a b Machiela MJ, Grünewald TG, Surdez D, Reynaud S, Mirabeau O, Karlins E, Rubio RA, Zaidi S, Grossetete-Lalami S, Ballet S, Lapouble E, Laurence V, Michon J, Pierron G, Kovar H, Gaspar N, Kontny U, González-Neira A, Picci P, Alonso J, Patino-Garcia A, Corradini N, Bérard PM, Freedman ND, Rothman N, Dagnall CL, Burdett L, Jones K, Manning M, Wyatt K, Zhou W, Yeager M, Cox DG, Hoover RN, Khan J, Armstrong GT, Leisenring WM, Bhatia S, Robison LL, Kulozik AE, Kriebel J, Meitinger T, Metzler M, Hartmann W, Strauch K, Kirchner T, Dirksen U, Morton LM, Mirabello L, Tucker MA, Tirode F, Chanock SJ, Delattre O (August 9, 2018). Scaplehorn N (ed.). "Genome-wide association study identifies multiple new loci associated with Ewing sarcoma susceptibility". Nature Communications. 9 (1:3184). Nature Portfolio: 3184. Bibcode:2018NatCo...9.3184M. doi:10.1038/s41467-018-05537-2. PMC 6085378. PMID 30093639.
  6. ^ "Primary Bone Cancer". www.cancer.gov. National Cancer Institute. 6 December 2018. Retrieved 25 August 2023.
  7. ^ Brown JM, Rakoczy K, Tokson JH, Jones KB, Groundland JS (2022-01-01). "Ewing sarcoma of the pelvis: Clinical features and overall survival". Cancer Treatment and Research Communications. 33: 100634. doi:10.1016/j.ctarc.2022.100634. ISSN 2468-2942. PMID 36126512. S2CID 252266945.
  8. ^ "Ewing's sarcoma". Whonamedit. Retrieved 4 February 2019.
  9. ^ Ewing J (September 2006). "The Classic: Diffuse endothelioma of bone. Proceedings of the New York Pathological Society. 1921;12:17". Clinical Orthopaedics and Related Research. 450: 25–7. doi:10.1097/01.blo.0000229311.36007.c7. PMID 16951641.
  10. ^ Brown JM, Rakoczy K, Tokson JH, Jones KB, Groundland JS (2022-01-01). "Ewing sarcoma of the pelvis: Clinical features and overall survival". Cancer Treatment and Research Communications. 33: 100634. doi:10.1016/j.ctarc.2022.100634. ISSN 2468-2942. PMID 36126512. S2CID 252266945.