Long-term results from the first UKCCSG Ewing's tumour study (ET-1)

Craft, A W; Cotterill, SJ; Ja, Bullimore; Pearson, Denise A.

doi:10.1016/s0959-8049(97)00043-9

Cited by 162 publications

(61 citation statements)

References 13 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

111

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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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“…An EFS of 70% was achieved for patients with localized disease, and OS reached 80%. [4][5][6][7][8][17][18][19][20][21] The outcome of metastatic patients remains dismal, especially for patients with bone and BM involvement. [4][5][6][9][10][11] Rodriguez Galindo et al 23 suggested risk stratification, taking into account simple clinical features, such as age, stage, tumor volume, localization and histological response to chemotherapy, to modulate treatment intensification in different subgroups.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3] In the past decade, ESFT outcome was improved largely through a multidisciplinary strategy based on surgery, radiotherapy and intensified chemotherapy regimens, [4][5][6] whereas large studies have identified risk factors associated with the worse prognosis. [4][5][6][7][8] Since the late 1980s, high-dose chemotherapy (HDC), followed by stem-cell rescue, has been proposed to improve the outcome of patients with poor-prognosis ESFT. Although many studies have been published, the role of HDC in ESFT remains unclear.…”

Section: Introductionmentioning

confidence: 99%

Toxicity of high-dose chemotherapy with etoposide, thiotepa and CY in treating poor-prognosis Ewing's sarcoma family tumors: the experience of the Bambino Gesù Children's Hospital

Ilari

Ioris

Milano³

et al. 2010

Bone Marrow Transplant

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We report the toxicity of high-dose chemotherapy (HDC) based on etoposide, thiotepa and CY (ETC) in children with poor-prognosis Ewing's sarcoma family tumors (ESFTs). A total of 26 patients with high-risk ESFT (metastasis or axis localization or tumor volume 4200 ml or necrosis o95%) were reviewed. The conditioning was based on etoposide (600 mg/m 2 ), thiotepa (750 mg/m 2 ) and CY (120 mg/kg) followed by autologous BM or PBSC rescue. The conditioning regimen was well tolerated, without any toxic deaths. The median time from transplant to a neutrophil count of 40.5 Â 10 9 /l was 10 days (range 6-27) and 22.5 days (range 9-114) for a plt count of 450 Â 10 9 /l. Oral mucositis was recorded in 20 patients, grade 1/2 in 19 and grade 3 in the last patient. Diarrhea grade 1/2 was recorded in four patients and grade 1/2 liver toxicity in four patients. Sepsis was documented in four cases and skin toxicity in three. Lung and tubular toxicity, respectively, were reported in one patient each. We conclude that the ETC regimen presented a limited and manageable toxicity. Further studies would confirm the role of ETC in high-risk ESFT.

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“…For ES involving the extremities, pelvis or non-axial skeleton, traditional treatment has consisted of induction chemotherapy after biopsy, followed by surgery [7, 21, 22, 23, 24]. This strategy has rarely been tried for primary ES of the skull largely because children frequently present with large lesions, raised intracranial pressure and impending neurological deficits demanding urgent neurosurgical intervention.…”

Section: Discussionmentioning

confidence: 99%

“…This strategy has rarely been tried for primary ES of the skull largely because children frequently present with large lesions, raised intracranial pressure and impending neurological deficits demanding urgent neurosurgical intervention. Effective chemotherapy for ES at any site includes combinations of agents such as ifosfamide, etoposide, vincristine, doxorubicin, cyclophosphamide and actinomycin D. The addition of chemotherapy in the treatment of ES has improved the rates of long-term survival, from 5–10% to 50–60% [7, 21]. Originally used as adjuvant therapy, chemotherapy is currently indispensable as first-line treatment because of its local and systemic efficacy [7, 21, 24, 25].…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing’s Sarcoma of the Skull in Children

et al. 1999

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Ewing’s sarcoma (ES) of the skull is rare. Herein, we present 2 cases of ES that involved the cranium in young children. In one case, the lesion originated in the petrous temporal bone; in the other, the frontal bone. Both children were acutely compromised neurologically by signs and symptoms of raised intracranial pressure. In both cases, radiographs revealed massive tumors affecting the skull. Neurosurgical resection of the tumor was undertaken in both instances, and the diagnosis of ES was confirmed by immunohistochemistry, cytogenetic analysis (translocation 11;22), spectral karyotyping and RT-PCR (demonstration of a EWS/FLI1 fusion transcript). Following aggressive surgical resection, both children received intensive chemotherapy. No child has received radiation therapy. One child is alive and well 8 years after diagnosis without any evidence of residual disease. The other is currently undergoing chemotherapy for her tumor. The principles involved in the management of children with cranial-based ES are discussed. These 2 cases serve to illustrate the fact that even children with massive ES tumors of the cranium may be salvaged with aggressive combination therapy.

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Long-term results from the first UKCCSG Ewing's tumour study (ET-1)

Cited by 162 publications

References 13 publications

Analysis of prognostic factors in ewing sarcoma family of tumors

Analysis of prognostic factors in ewing sarcoma family of tumors

Toxicity of high-dose chemotherapy with etoposide, thiotepa and CY in treating poor-prognosis Ewing's sarcoma family tumors: the experience of the Bambino Gesù Children's Hospital

Primary Ewing’s Sarcoma of the Skull in Children

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