Ewing’s sarcoma family of tumors (ESFT)

Includes Ewing’s sarcoma. Peripheral neuroectodermal tumor (PNET), extraosseous Ewing’s sarcoma and Ewing’s sarcoma of the chest wall (Askin’s tumor).

Tumors of neural crest derivation described as small round cell tumors.

Characterized by balanced chromosomal translocation between 5’half of the EWS gene (22q12) and the 3’half of members of the ETS family of transcription factors indicating that ESFT represents a single malignant entity.

The fusion gene maintains the malignant phenotype in Ewing’s tumor cells and may also modulate gene expression at the RNA level.

Molecular hallmark is the nonrandom translocation of the EWS gene.

90% of cases have translocation of the t(11;22)EWS-FLII, which fuses the N-terminal domain of EWS with the DNA domain of the FLII.

High grade, locally advanced tumors with strong propensity for distant metastases.

Can arise anywhere in the body, including bone or soft tissue.

Characterize molecularly by rearrangements of the EWS gene.

Approximately 70% of patients with localized ESFT or cured with combined multi modality therapy including chemotherapy, radiotherapy, surgery, or all.

Approximately 20% of patients with ESFT present with metastatic disease, and less than 30% of such patients all long-term survivors (Balamuth NJ et al).

Most Ewing tumor cells have increased expression of insulin like growth factor1 (IGF-1R)or IGF-2ligand.

Likelihood of cure for localized Ewing’s sarcoma family use 70%.

The survival rate for patients with metastatic Ewing’s sarcoma family tumors at diagnosis is less than 30%.

Ewing’s sarcoma is the second most common bone tumor in adolescents and young adults.

Treatment for Ewing’s sarcoma consists of at least six cycles of neoadjuvant chemotherapy, followed by local management.

Surgery is the pref2242ed local control, but radiation therapy is used to complete local control when surgery is unachievable.

Following surgery/radiation, another 8 cycles of adjuvant chemotherapy can be administered and the lesions chosen vary depending upon previous pathological response.

Current standard therapy includes vincristine, dactinomycin, doxorubicin, and cyclophosphamide with the addition of ifosfamide and etoposide (Grier HE).

Adults have worse prognosis compared to childhood disease.

Second line salvage therapy includes agents such as topotecan, cyclophosphamide, temozolomide and irinotecan.

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