A multidisciplinary study investigating radiotherapy in Ewing’s sarcoma: end results of POG #8346

Donaldson, Sarah S.; Torrey, Margaret J.; Link, Michael P.; Glicksman, Arvin S.; Gilula, Louis A.; Laurie, Fran; Manning, John T.; Neff, James R.; Reinus, William R.; Thompson, Elizabeth I.; Shuster, Jonathan J.

doi:10.1016/s0360-3016(98)00191-6

Cited by 191 publications

(128 citation statements)

References 27 publications

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“…[11][12][13][14][15][16] Similar to the ES79 protocol, surgery was seldom used, and RT alone was used in [75% of the patients. 11,12,15,[17][18][19] The ES87 protocol built on the evidence indicating the efficacy of IE, 20 and incorporated this pair into a VACD backbone. 7 However, using similar local control approaches to the ES79 protocol, the addition of IE in the ES87 protocol did not appear to improve the outcomes.…”

Section: Advances In the Treatment Of Esftmentioning

confidence: 99%

Analysis of prognostic factors in ewing sarcoma family of tumors

Rodríguez‐Galindo

Liu

Krasin

et al. 2007

Cancer

209

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BACKGROUND.Advances in systemic and local therapies have improved outcomes for patients with the Ewing sarcoma family of tumors (ESFT). As new treatments are developed, a critical review of data from past treatment eras is needed to identify clinically relevant risk groups.METHODS.The authors reviewed the records of 220 patients with ESFT who were treated on protocols at St. Jude Children's Research Hospital from 1979 to 2004. Two treatment eras were defined. Factors predictive of outcome were analyzed to identify distinct risk groups.RESULTS.The median age at diagnosis was 13.7 years (range, 1.1–25.2 years). Metastatic disease was associated with tumors measuring >8 cm (P = .002) and axial location (P = .014). The 5‐year overall survival (OS) estimate (63.5% ± 3.5%) did not appear to differ by protocol. Tumor stage and size were found to be the only independent predictors of outcome. Treatment era and type of local control therapy were found to influence the outcome of patients with localized disease. Four risk groups were defined: favorable risk (age <14 years with localized, nonpelvic tumors), intermediate risk (localized, age ≥14 years, or pelvic tumors), unfavorable‐pulmonary (isolated lung metastases), and unfavorable‐extrapulmonary (extrapulmonary metastases). The 5‐year OS estimates for these groups were 88.1% ± 4.4%, 64.9% ± 5.2%, 53.8% ± 9.4%, and 27.2% ± 7.3%, respectively (P < .001). The incidence of therapy‐related leukemia was significantly higher during the second treatment era, when more intensified regimens were used (6.1% ± 2.7% vs 0% ± 0%; P = .005).CONCLUSIONS.Risk stratification schemes such as this should be used to prospectively evaluate novel risk‐based therapies. Studies of biologic pathways may help to refine this model. Cancer 2007. © 2007 American Cancer Society.

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Section: Advances In the Treatment Of Esftmentioning

confidence: 99%

Analysis of prognostic factors in ewing sarcoma family of tumors

Rodríguez‐Galindo

Liu

Krasin

et al. 2007

Cancer

209

111

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“…2 Currently, in the field of multimodality therapy, event-free survival (EFS) rates have increased from 10% to 470% in localised disease. 3,4 Isolated pulmonary metastatic disease is associated with an EFS rate of 29-52%. 5 Although a slight improvement has been achieved with single high-dose chemotherapy (HDC) regimen, the outcome of patients with primary disseminated multifocal metastatic Ewing's sarcoma (PDMES) remains dismal.…”

Section: Introductionmentioning

confidence: 99%

Tandem high-dose chemotherapy strategy as first-line treatment of primary disseminated multifocal Ewing sarcomas in children, adolescents and young adults

Loschi

Dufour

Oberlin

et al. 2015

Bone Marrow Transplant

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The prognosis of primary disseminated multifocal metastatic Ewing's sarcoma (PDMES) is poor even if a slight improvement has been achieved with high-dose alkylating agent-containing chemotherapy. To enhance treatment efficacy, we assessed the feasibility, safety and efficacy of a tandem high-dose chemotherapy (HDC) regimen. In a single institution, patients with PDMES received six courses of vincristine/ifosfamide/doxorubicin/etoposide induction therapy, followed by high-dose thiotepa, and then melphalan-busulfan, 8 weeks apart. Surgical resection of primary tumour was carried out between the two HDC regimens and 70 days after the last HDC regimen for post-operative radiotherapy or irradiation alone. From October 2002 to 2009, 13 of the 18 consecutive patients with PDMES (72%) received the full treatment programme. The other five patients experienced early progression and died. Among the 13 patients, 11 relapsed after the end of the treatment programme within 6 months (2.2-11.9) from end of therapy. Only two patients are still alive in first complete remission after 9 years. The 3-year event-free survival (EFS) and overall survival (OS) rates were 11 and 22%, respectively. The median EFS and OS duration from the diagnosis were 13.4 and 17.3 months, respectively. Neither major complications nor treatment-related death occurred. The tandem-HDC regimen was feasible, with expected side effects, but it did not improve the outcome of patients with PDMES. INTRODUCTIONEwing sarcoma (ES) is the second most common primary malignant bone tumour in children, adolescents and young adults after osteosarcoma. 1 ES is characterised by a specific translocation t(11;22) and transcript EWS-Fli1. 2 Currently, in the field of multimodality therapy, event-free survival (EFS) rates have increased from 10% to 470% in localised disease. 3,4 Isolated pulmonary metastatic disease is associated with an EFS rate of 29-52%. 5 Although a slight improvement has been achieved with single high-dose chemotherapy (HDC) regimen, the outcome of patients with primary disseminated multifocal metastatic Ewing's sarcoma (PDMES) remains dismal. The 3-year EFS rate was 27% with the chemotherapy regimen in the R3 arm of the EuroEWING99 (EE99) trial. 6 This regimen comprised a six-course induction chemotherapy of vincristine/ifosfamide/doxorubicin/ etoposide (VIDE), then one course of vincristine/(d)actinomycin/ ifosfamide (VAI) and a single-HDC regimen by busulfan-melphalan (Bu/Mel), as consolidation therapy, followed by autologus peripheral haematopoietic progenitor (APHP) rescue. Prognostic factors (age, initial tumour volume, number of bone metastases, bone marrow and lung involvement) had allowed clinicians to define a prognostic score in this population. 6,7 The 3-year EFS rate was 50, 25 and 10% for a score of o 3, from 3 to 5 and for a score ⩾ 5, respectively. 6 The benefit of a myeloablative regimen in terms of outcome has been demonstrated in several tumour types with high sensitivity to HDC (for example, high-risk neuroblastomas). 8 In multimeta...

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“…[22][23][24][25][26][27][28][29] But radiation therapy also carries risk of skin necrosis, decrease in immune status, secondary malignancy, etc. Complication rates for preoperative radiotherapy reported by various authors range from 10% to 41%.…”

Section: Significance Of Radiotherapymentioning

confidence: 99%