Ewing's sarcoma
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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
- primary sites include:
- lower extremity (41%)
- upper extremity (9%)
- chest wall (16%)
- pelvis (26%)
- spine (6%)
- skull (2%)[4]
- constitutional symptoms common
- pain not relieved by NSAIDs
Laboratory
Radiology
Complications
- 25% of tumors metastatic at time of diagnosis[4]
Management
- see ref[4]
- multidrug chemotherapy
- protocols all include:
- nost protocols use cyclophosphamide as well
- certain protocols incorporate dactinomycin
- surgery &/or radiation therapy
More general terms
Additional terms
- 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)
- 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)
References
- ↑ Wikipedia: Ewing' sarcoma http://en.wikipedia.org/wiki/Ewing's_sarcoma
- ↑ Iwamoto Y Diagnosis and treatment of Ewing sarcoma Jpn J Clin Oncol 2007, 37:79 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/17272319 <Internet> http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17272319.
- ↑ Burt M et al Medical tumors of the chest wall. Solitary plasmacytoma and Ewing sarcoma J Thorac Cardiovasc Surg 105: 89 PMID: https://www.ncbi.nlm.nih.gov/pubmed/8419714
- ↑ 4.0 4.1 4.2 4.3 4.4 ARUP Consult: Ewing Sarcoma The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/ewing-sarcoma
- ↑ 5.0 5.1 NEJM Knowledge+ Question of the Week. Jan 8, 2018 https://knowledgeplus.nejm.org/question-of-week/4923/
- ↑ 6.0 6.1 Kaste SC. Imaging pediatric bone sarcomas. Radiol Clin North Am 2011 Jul; 49:749. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21807172 Free PMC Article
- ↑ Arndt CA, Crist WM. Common musculoskeletal tumors of childhood and adolescence. N Engl J Med 1999 Jul 29; 341:342. <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/10423470 <Internet> http://www.nejm.org/doi/full/10.1056/NEJM199907293410507
- ↑ 8.0 8.1 Riggi N, Suva ML, Stamenkovic I Ewing's Sarcoma. N Engl J Med 2021; 384:154-164. Jan 14. PMID: https://www.ncbi.nlm.nih.gov/pubmed/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 - ↑ National Cancer Institute - Ewing's Family of Tumors Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/ewings/healthprofessional
Patient information
Ewing sarcoma patient information