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Ewing sarcoma

Friday 21 May 2004

Digital cases (Digital slides)

- HPC:31 : Ewing sarcoma
- HPC:132 : Ewing sarcoma
- HPC:182 : Ewing sarcoma
- HPC:376 : EWSR1-FLI1+ bone-forming Ewing sarcoma (EWSR1-FLI1+ small cell osteosarcoma)

Variants

- epithelioid Ewing sarcoma
- spindle cell Ewing sarcoma
- atypical pleomorphic extraosseous Ewing sarcoma
- large cell Ewing sarcoma
- neuro-differentiated Ewing sarcoma (pPNET, peripheral primitive neuroectodermal tumor)
- bone-forming Ewing sarcoma (small cell osteosarcoma)

Localization

- skeletal Ewing sarcoma (osseous Ewing sarcoma)
- extraskeletal Ewing sarcoma (extraosseous Ewing sarcoma)

  • renal Ewing sarcoma

Immunochemistry

- CD99+ (MIC2+)
- FLI1+

Variants

- post-chemotherapy neuroblastoma-like differentiation in pPNET (#16944972#)
- Ewing sarcoma with osteoid formation (#15188147#)

Cytogenetics and molecular biology

Ewing family tumors are molecularly characterized by expression of chimeric transcripts generated by specific chromosomal translocations, most commonly involving fusion of the EWS gene to a member of the ETS family of transcription factors (including FLI1, ERG, ETV1, E1AF, and FEV).

Approximately 85% of reported cases of Ewing sarcoma bear an EWS-FLI1 fusion.

In rare cases, FUS can substitute for EWS, with translocation t(16;21)(p11;q24) producing a FUS-ERG fusion with no EWS rearrangement.

- EWSR1 rarrangements

  • t(11;22)(q24;q12) (EWSR1/FLI1 fusion gene) (85%) (11q24 and 22q12)
  • t(21;22)(q22;q12) (EWSR1/ERG fusion gene) (21q22 and 22q12)
  • t(2;22)(q36; q12) (EWSR1/FEV fusion gene) (2q36 and 22q12)
  • t(17;22)(q21;q12) (EWSR1/ETV4 fusion gene) (17q21 and 22q12)

- FUS rearrangements

  • t(16;21)(p11;q22) (FUS/ERG fusion gene) (16p11 and 22q12)
  • t(2;16)(q35;p11) (FUS/FEV fusion gene) (#17620387#)

Chromosomal imbalance (CGH)

Gains 1q+ 8+ 12+

See also

- Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET)or "Ewing family of tumors" (EFTs)

Portfolio

  • Ewing sarcoma
  • Ewing sarcoma
  • Ewing sarcoma
  • Ewing sarcoma: CD99 positivity
  • Ewing sarcoma: CD99 positivity
  • Ewing sarcoma: CD99 positivity
  • Ewing sarcoma of the fibula
  • Femoral Ewing sarcoma
  • Femoral Ewing sarcoma
  • Femoral Ewing sarcoma
  • Femoral Ewing sarcoma
  • Femoral Ewing sarcoma
  • Ewing sarcoma in spongious bone
  • Ewing sarcoma in spongious bone
  • Nodal metastasis of an Ewing sarcoma at a pulmonary hilus
  • Costal Ewing sarcoma
  • Costal Ewing sarcoma