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Ewing's sarcoma

Epidemiology: - rare, 1 case per 1.5 million population - 10-fold more common in European vs African origin - primarily children & adolescents, mean age at diagnosis = 15 years - male/female ratio of 1.6:1 Pathology: - malignant round-cell tumor - malignant cells can have characteristics of both mesodermal & ectodermal origin - malignant cells are found in the bone or in soft tissue - most commonly involves the pelvis & proximal long bone - extraosseous Ewing's sarcoma occurs much more frequently in adults than in children [8] Genetics: - translocations involving chromosomes 22q12 & 11q24 frequent - chromosomal translocation t(9;22)(q22-31;q11-12) involving NR4A3 with EWS - chromosomal translocation t(7;22)(p22;q12) involving ETV1 with EWS is a cause of Ewing sarcoma - chromosomal translocation t(2;21;22)(q23;q22;q12) involving FEV with EWSR1 is found in Ewing tumors; translocation forms a EWSR1-FEV fusion protein with potential oncogenic activity - EWS fuses with FLI1, ERG, ETV1 - ETAA1 expressed at high levels Clinical manifestations: 1) primary sites include: a) lower extremity (41%) b) upper extremity (9%) c) chest wall (16%) d) pelvis (26%) e) spine (6%) f) skull (2%) [4] 2) constitutional symptoms common 3) pain not relieved by NSAIDs Laboratory: - see ARUP consult [4] Radiology: - radiograph - sclerotic lesion - onion skin periosteal bone reaction [5,6] - usually affects shaft of the bone Complications: - 25% of tumors metastatic at time of diagnosis [4] Management: 1) see ref [4] 2) multidrug chemotherapy a) protocols all include: - vincristine, doxorubicin, ifosfamide, & etoposide b) nost protocols use cyclophosphamide as well c) certain protocols incorporate dactinomycin 3) surgery &/or radiation therapy

Interactions

disease interactions

Related

CD99; T-cell surface glycoprotein E2; E2 antigen; protein MIC2; 12E7 (CD99, MIC2, MIC2X, MIC2Y) chromosomal translocation t(11;22)(q24;q12) (Ewing's sarcoma) chromosomal translocation t(21;22)(q22;q12) erg proto-oncogene protein Et translocation variant 1; ER81 protein; Ets-related variant 1 (ETV1) Ewing's tumor-associated antigen 1 (Ewing's tumour-associated antigen 16, ETAA1, ETAA16) Friend leukemia integration 1 transcription factor; Fli-1 proto-oncogene protein; ERGB transcription factor (FLI1) nuclear hormone receptor NOR-1 (orphan nuclear receptor NR4A3, neuron-derived orphan receptor 1, mitogen-induced nuclear orphan receptor,nuclear receptor subfamily 4 group A member 3, NR4A3, CHN, CSMF, NOR1, TEC) RNA-binding protein EWS; EWS oncogene; Ewing sarcoma breakpoint region 1 protein (EWSR1, EWS) SH2 domain-containing protein 1B; EWS/FLI1-activated transcript 2; EAT-2 (SH2D1B, EAT2)

General

bone cancer Ewing's sarcoma family of tumors soft tissue sarcoma (STS)

Database Correlations

OMIM 133450

References

  1. Wikipedia: Ewing' sarcoma http://en.wikipedia.org/wiki/Ewing's_sarcoma
  2. Iwamoto Y Diagnosis and treatment of Ewing sarcoma Jpn J Clin Oncol 2007, 37:79 PMID: 17272319 http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17272319.
  3. Burt M et al Medical tumors of the chest wall. Solitary plasmacytoma and Ewing sarcoma J Thorac Cardiovasc Surg 105: 89 PMID: 8419714
  4. ARUP Consult: Ewing Sarcoma The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/ewing-sarcoma
  5. NEJM Knowledge+ Question of the Week. Jan 8, 2018 https://knowledgeplus.nejm.org/question-of-week/4923/
  6. Kaste SC. Imaging pediatric bone sarcomas. Radiol Clin North Am 2011 Jul; 49:749. PMID: 21807172 Free PMC Article
  7. Arndt CA, Crist WM. Common musculoskeletal tumors of childhood and adolescence. N Engl J Med 1999 Jul 29; 341:342. PMID: 10423470 http://www.nejm.org/doi/full/10.1056/NEJM199907293410507
  8. Riggi N, Suva ML, Stamenkovic I Ewing's Sarcoma. N Engl J Med 2021; 384:154-164. Jan 14. PMID: 33497548 https://www.nejm.org/doi/full/10.1056/NEJMra2028910 - Rothaus C Ewing's Sarcoma. NEJM Resident 360. Jan 13, 2021 https://resident360.nejm.org/clinical-pearls/ewing-s-sarcoma
  9. National Cancer Institute - Ewing's Family of Tumors Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/ewings/healthprofessional