Identification of Patients With Localized Ewing Sarcoma at Higher Risk for Local Failure: A Report From the Children's Oncology Group

Ahmed, Safia K.; Randall, R. Lor; DuBois, Steven G.; Harmsen, William S.; Krailo, Mark; Marcus, Karen J.; Janeway, Katherine A.; Geller, David S.; Sorger, Joel; Womer, Richard B.; Granowetter, Linda; Grier, Holcombe E.; Görlick, Richard; Laack, Nadia N.

doi:10.1016/j.ijrobp.2017.08.020

Cited by 45 publications

(42 citation statements)

References 38 publications

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“…RT alone is an acceptable treatment for local control as well, but some evidence has suggested that surgery improved outcomes [32]. Ahmed et al showed that patients who underwent surgical resection, whether wide or less than wide, had improved survival compared to patients who did not have surgery [33]. In accordance with the prior literature, our study did not find an association between R0 surgical resection and overall survival [29, 34–37].…”

Section: Discussionsupporting

confidence: 88%

Role of Radiation Therapy in Adult Extraskeletal Ewing’s Sarcoma Patients Treated with Chemotherapy and Surgery

Saiz

Gingrich

Canter

et al. 2019

Sarcoma

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Radiation therapy (RT) is advocated in the multimodal treatment of high-grade soft tissue sarcoma (STS), but its role may be less clear in chemotherapy-sensitive STS such as extraskeletal Ewing sarcoma (EES). The purpose of this study was to determine the role of RT on overall survival (OS) in localized EES adult patients treated with chemotherapy and surgery. Adult patients diagnosed with EES and reported to the National Cancer Database from 2004 to 2014 were evaluated. All patients were treated with surgical resection. Patient demographics, tumor characteristics, treatments received, resection margins, and survival were examined for the 232 patients identified. Using multivariate analysis and Cox proportional hazard analysis, predictors of OS were determined. In the overall cohort, 40 percent of patients received RT and 78 percent received chemotherapy, with 31 percent receiving both. The addition of RT to the patients receiving surgery + chemotherapy did not improve OS (p<0.05). Twenty-four percent of patients who achieved R0 resection after surgery still received RT without any improvement in OS. Patients treated at community cancer centers were more likely to receive additional RT compared with Comprehensive Cancer Centers (p<0.05). In adult EES patients with localized disease treated with chemotherapy and surgery, the addition of RT does not improve overall survival.

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Section: Discussionsupporting

confidence: 88%

Role of Radiation Therapy in Adult Extraskeletal Ewing’s Sarcoma Patients Treated with Chemotherapy and Surgery

Saiz

Gingrich

Canter

et al. 2019

Sarcoma

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“…The local recurrence rate in our study is comparable to what has been found in some other studies [4, 10, 13–15, 17]. However, the rate is higher than in the Children's Oncology Group study by Ahmed et al which reported a 3.6% 5 year local recurrence rate, all local recurrences occurring in the group treated by definitive RT [30]. The excellent local control observed among patients treated with excisional surgery in our study may be attributed to improvements in chemotherapy rather than to the surgical treatment of the primary tumor.…”

Section: Discussionsupporting

confidence: 87%

Ewing sarcoma of the mobile spine; predictive factors for survival, neurological function and local control. A Scandinavian sarcoma group study with a mean follow-up of 12 years

Hesla

Bruland

Jebsen

et al. 2019

Journal of Bone Oncology

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Object: Many patients with Ewing sarcoma (ES) of the mobile spine present with neurologic symptoms leading to emergency decompressive surgery. Only rarely is optimal treatment involving neo-adjuvant chemotherapy followed by en bloc excision possible. The purpose of this study was to study treatment, neurologic and oncologic outcome in patients with ES of the mobile spine.Methods: Twenty-four patients diagnosed between 1986 and 2012 were identified through the Scandinavian Sarcoma Group registry. Charts were reviewed in order to assess details in patient characteristics, neurologic status, treatment and outcome. Prognostic factors were analyzed with respect to local control, disease-free survival and overall survival.Results: Neurologic symptoms were frequently observed at presentation, being present in 19/23 patients with documented neurologic status. Most (13/19) patients had a complete neurologic recovery regardless of whether or not emergency decompressive surgery was performed. The majority (18/24) of patients were treated with definitive radiotherapy. However, only 9/17 received the recommended dose of  ≥  50.4 Gy. The disease-free and overall survival rates at 10 years were 48% and 57%, respectively. The local recurrence rates were 19% and 27% at 5 and 10 years, respectively. Only year of diagnosis, categorized into periods with significant changes in chemotherapy protocols, was a significant factor for local recurrence, but there was a trend (p = 0.06) for an increased risk of a local recurrence if emergency decompressive surgery was performed.Conclusion: Patients with ES of the mobile have a relatively favorable prognosis. Nonetheless, local recurrence rate is high for this group of patients for which local treatment mainly relies on definitive radiotherapy.Emergency decompressive surgery may increase the risk for local recurrence.

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“…A review of outcomes for EWS in the National Cancer Database found a decreased 5-year survival in patients with radiation alone (52.5%) compared to patients treated with surgery alone (77.2%). 22 A further analysis of patients treated in a Brazilian collaborative study also found that patients treated with radiation alone for local control had considerably worse outcomes with only a 17.8% survival at 5 years when treated with radiation alone. 23 These results should be considered when evaluating the potential for complete resection of intracranial lesions and consideration for more aggressive surgery including the risks and benefits of cranioplasty.…”

Section: Discussionmentioning

confidence: 96%

Intracranial Ewing sarcoma: four pediatric examples

et al. 2017

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BackgroundEwing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis.ProcedureNeurosurgical Department records, spanning 21 years (1995–2016), were reviewed to identify patients. Demographics, clinical history, pathological/genetic features, and clinical course were retrieved from the medical record and personal files of the authors.ResultsFour patients, one male and three females, age 5 to 16 years, were identified. One presented in extremis from a large lesion, two with soft tissue masses, and the fourth as an incidental finding after being involved in a motor vehicle collision. Three had clear bony involvement: a 10-year-old girl with a large left temporal lesion had clear origin in the skull, with spiculated calcified striations throughout the mass; a 9-year-old girl presented with a bony left petrous apex mass; and a 16-year-old girl presented with a left temporal mass with extension to the dura and underlying bone erosion. Only the 5-year-old boy had a large left frontoparietal mass traversing the falx with no bony contact. All four tumors manifested the diagnostic EWSR1 mutation and were treated with an Ewing sarcoma regimen. Outcomes were variable, with one patient showing progressive metastatic disease and death 3 years after presentation, one patient with disease-free survival 10.5 years after completion of therapy, and one alive and well at the completion of therapy 1 year after diagnosis. One patient completed therapy recently with post-therapy scans showing no evidence of disease.ConclusionTesting for the EWSR1 mutation confirms the diagnosis of Ewing sarcoma and excludes other types of embryonal CNS tumors. Long-term disease-free survival is possible with adherence to the appropriate therapeutic regimen after gross surgical resection.

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Identification of Patients With Localized Ewing Sarcoma at Higher Risk for Local Failure: A Report From the Children's Oncology Group

Cited by 45 publications

References 38 publications

Role of Radiation Therapy in Adult Extraskeletal Ewing’s Sarcoma Patients Treated with Chemotherapy and Surgery

Role of Radiation Therapy in Adult Extraskeletal Ewing’s Sarcoma Patients Treated with Chemotherapy and Surgery

Ewing sarcoma of the mobile spine; predictive factors for survival, neurological function and local control. A Scandinavian sarcoma group study with a mean follow-up of 12 years

Intracranial Ewing sarcoma: four pediatric examples

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