A Retrospective Multicentric Study of Ewing Sarcoma Family of Tumors in Patients Older Than 50: Management and Outcome

Rochefort, Pauline; Italiano, Antoîne; Laurence, Valérie; Penel, Nicolas; Lardy-Cléaud, Audrey; Mir, Olivier; Chevreau, Christine; Bertucci, François; Bompas, Emmanuelle; Chaigneau, L.; Lévy, Dominique; Ryckewaert, Thomas; Dumont, Sarah; Méeus, Pierre; Ranchère, Dominique; Blay, Jean‐Yves; Cassier, Philippe

doi:10.1038/s41598-017-17733-z

Cited by 11 publications

(11 citation statements)

References 41 publications

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“…Our observation that extraosseous ESHN was more common in adult patients is in agreement with previous reports of ES arising from other sites, 26 though the underlying mechanism for this relationship is not clear. ES in older patients has been reported to bear a higher mutational burden than tumors from younger patients.…”

Section: Discussionsupporting

confidence: 91%

Clinical characteristics and treatment‐associated survival of head and neck Ewing sarcoma

et al. 2019

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Objectives Ewing sarcoma of the head and neck (ESHN) is a rare malignancy for which limited data exists. Herein we examine anatomic distribution of ESHN, demographic characteristics, and multi‐modal therapy as potential determinants in the overall survival (OS) of patients with ESHN. Methods A retrospective study of the National Cancer Database (NCDB) from 2004–2016 was performed. A multivariate Cox regression and univariate Kaplan‐Meier survival analyses were conducted. Results We identified 284 patients with ESHN eligible for demographic analysis and 223 for survival analysis. Approximately half (50.3%) of patients with ESHN were under the age of 18. Pediatric patients (<18) were less likely to present with distant metastases (≤8.0% vs. 9.5–19.0%, P = .006) and more likely to present with osseous disease (90.2% vs. 75.2%, P = .001) than adults. Adult age (HR: 2.727, 95% CI, 1.381–5.384, P = .004) and distant metastatic disease at the time of presentation (HR: 8.161, 95% CI, 2.922–22.790, P < .001) were independently associated with worse OS. The addition of local therapy (either surgery, radiotherapy, or both) to chemotherapy was not associated with improved survival when compared to treatment with chemotherapy alone. Conclusion Predictors of OS in patients with ESHN included age < 18 years and non‐metastatic disease at the time of diagnosis. Tumor site of origin (osseous vs. extraosseous primary) or the addition of local therapy to chemotherapy had no impact on OS. Our analysis suggests that chemotherapy serves as the primary modality in treating ESHN, while further study of this rare malignancy is required to discern the utility of combined systemic and local therapy. Laryngoscope, 130:2385–2392, 2020

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Section: Discussionsupporting

confidence: 91%

Clinical characteristics and treatment‐associated survival of head and neck Ewing sarcoma

et al. 2019

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“…Numerous studies have compared the outcome of pediatric and adult cases of ES, and the majority show that the behavior of this tumor in adults is comparable with the pediatric population. 2,[23][24][25][26][27][28][29][30][31] In our series, approximately one third of patients (15 of 43, 35%) developed metastases, and 7 of those had metastasis at the time of presentation, comparable with previous reports. 2,27 Furthermore, it appears that extraskeletal and skeletal tumors in our study had similar outcomes, also paralleling existing data in the literature.…”

Section: Discussionsupporting

confidence: 90%

Ewing Sarcoma in Older Adults: A Clinicopathologic Study of 50 Cases Occurring in Patients Aged ≥40 Years, With Emphasis on Histologic Mimics

et al. 2019

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Objective. We explore the clinicopathologic features of Ewing sarcoma (ES) presenting in older adulthood. Methods. Cases of molecularly confirmed ES arising in patients aged ≥40 years were evaluated. Results. Fifty patients were identified (33 males/17 females; 41-86 years). The majority of tumors (41) arose at extraskeletal sites, while 9 were bone primaries. Twenty-eight cases showed nested architecture, while the remaining cases showed sheet-like growth. Tumor cytology was categorized as conventional (n = 39), crushed (n = 5), clear cell (n = 4), rhabdoid (n = 3), and epithelioid (n = 2). Fifty percent had necrosis, while rosettes were noted in 1 case. Immunostains performed ranged from 1 to 28 (median = 10). Follow-up (n = 43, 1-147 months) revealed 15 patients with metastasis. Conclusion. Although rare, ES should be considered in the differential diagnosis for round cell malignancies in older adult patients. In this cohort, ES is most often extraskeletal, and may show unusual morphologic features, closely simulating more common neoplasms in this age group.

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“…EwS constitutes the second most common bone-associated sarcoma in children, adolescents, and young adults after osteosarcoma [1][2][3]. However, EwS also occurs in soft tissue, although less frequently and predominantly in older patients [4,5].…”

Section: Introductionmentioning

confidence: 99%

High Specificity of BCL11B and GLG1 for EWSR1-FLI1 and EWSR1-ERG Positive Ewing Sarcoma

Orth¹,

Hölting²,

Dallmayer

et al. 2020

Cancers

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Ewing sarcoma (EwS) is an aggressive cancer displaying an undifferentiated small-round-cell histomorphology that can be easily confused with a broad spectrum of differential diagnoses. Using comparative transcriptomics and immunohistochemistry (IHC), we previously identified BCL11B and GLG1 as potential specific auxiliary IHC markers for EWSR1-FLI1-positive EwS. Herein, we aimed at validating the specificity of both markers in a far larger and independent cohort of EwS (including EWSR1-ERG-positive cases) and differential diagnoses. Furthermore, we evaluated their intra-tumoral expression heterogeneity. Thus, we stained tissue microarrays from 133 molecularly confirmed EwS cases and 320 samples from morphological mimics, as well as a series of patient-derived xenograft (PDX) models for BCL11B, GLG1, and CD99, and systematically assessed the immunoreactivity and optimal cut-offs for each marker. These analyses demonstrated that high BCL11B and/or GLG1 immunoreactivity in CD99-positive cases had a specificity of 97.5% and an accuracy of 87.4% for diagnosing EwS solely by IHC, and that the markers were expressed by EWSR1-ERG-positive EwS. Only little intra-tumoral heterogeneity in immunoreactivity was observed for differential diagnoses. These results indicate that BCL11B and GLG1 may help as specific auxiliary IHC markers in diagnosing EwS in conjunction with CD99, especially if confirmatory molecular diagnostics are not available.

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A Retrospective Multicentric Study of Ewing Sarcoma Family of Tumors in Patients Older Than 50: Management and Outcome

Cited by 11 publications

References 41 publications

Clinical characteristics and treatment‐associated survival of head and neck Ewing sarcoma

Clinical characteristics and treatment‐associated survival of head and neck Ewing sarcoma

Ewing Sarcoma in Older Adults: A Clinicopathologic Study of 50 Cases Occurring in Patients Aged ≥40 Years, With Emphasis on Histologic Mimics

High Specificity of BCL11B and GLG1 for EWSR1-FLI1 and EWSR1-ERG Positive Ewing Sarcoma

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