Deskriptive Epidemiologie der Ewing-Tumoren - Analysen der deutschen Patienten von (EI) CESS 1980-1997*

Hw, Hense; Ahrens, S.; Paulussen, Michael; Lehnert, Martin; Jürgens, H.

doi:10.1055/s-2008-1043799

Cited by 45 publications

(14 citation statements)

References 2 publications

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“…The only risk factor in our study that translated to significant PFS and OS difference was presence of metastasis (P = 0.002, 0.001) respectively. This is consistent with the evidence-based prognostic factor accepted by all authors [6] [18] [19].…”

Section: Discussionsupporting

confidence: 79%

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Prognosis and Outcome of Adult Patients with Ewing Sarcoma (Local Experience)

Ashour¹,

Shammari²,

Shafik³

et al. 2017

JCT

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Objective: Aim of this work is to assess clinical features, outcome and prognosis of adult patients diagnosed with Ewing Sarcoma (ES). Patients, Methods and Results: The records of 53 adult patients treated with Euro-Ewing protocol at Kuwait cancer control center (KCCC) over a period of 5 years were reviewed. Mean age was 26.9 ± 1.25 years. Thirty percent of patients presented with metastatic disease, and 65% of tumors were centrally located and 50% were ≥ 8 cm. All patients received initially VIDE protocol. 13.5% achieved complete remission (CR), and 57.7% achieved partial response (PR). Approximately 1/3 of patients underwent surgery which was adequate in 76% of them, and all patients received local radiotherapy. Post local treatment 56% of patients received VAC and 44% received VAI protocol. Mean treatment duration was 11 months ± 0.54. Median follow-up duration was 38.39 (33.49 -43.28) months At the end of follow up 20% of patients relapsed locally and 36% distally. Median PFS was 46.9 months (95% CI 41.42 -52.39), ); survivals at 3 and 5 years were 88%, 46% respectively. Conclusion: In this series metastases at presentation and wide surgical margins were the most important prognostic factors. Multimodality therapy is necessary for this rare disease.

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Section: Discussionsupporting

confidence: 79%

“…The exact reason of the poor prognostic effect of age is not clearly understood till now, however it may be due to the fact that most adult cases present with pelvic disease and other biological factories [5] [6].…”

mentioning

confidence: 99%

Prognosis and Outcome of Adult Patients with Ewing Sarcoma (Local Experience)

Ashour¹,

Shammari²,

Shafik³

et al. 2017

JCT

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“…Approximately half of all patients are between 10 and 20 years of age at the time of first diagnosis, making this highly undifferentiated tumor the second most common primary malignant bone tumor in children and adolescents (Grier, 1997;Denny, 1998;Kovar, 1998;de Alava and Gerald, 2000;Paulussen et al, 2001). The frequency of occurrence in children is in the range of 1-3 per million per year in the Western hemisphere; interestingly, Ewing's sarcoma is slightly more frequent in males than females as well as more frequent in people of European than Chinese or African origin (Fraumeni and Glass, 1970;Young and Miller, 1975;Li et al, 1980;Stiller and Parkin, 1996;Zucman-Rossi et al, 1997;Hense et al, 1999;Cotterill et al, 2000).…”

Section: Introductionmentioning

confidence: 96%

EWS–ETS oncoproteins: The linchpins of Ewing tumors

Janknecht

2005

Gene

153

120

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“…The rate of longterm survival is 50% to 70% at 5 years for patients with localized tumor but only 15% to 20% when pulmonary metastases are detected at diagnosis or not responding to therapy, or with disease relapse. Ewing's sarcoma is a highgrade neoplasm that accounts for approximately half of osteosarcoma cases in children, representing 2% of childhood cancers with a peak incidence at age 15 years (3)(4)(5). It is characterized by a rapid tumor growth and extensive bone destruction that can result in bone pain and pathologic fracture (6).…”

mentioning

confidence: 99%

Preclinical Evidence that Use of TRAIL in Ewing's Sarcoma and Osteosarcoma Therapy Inhibits Tumor Growth, Prevents Osteolysis, and Increases Animal Survival

Picarda

Lamoureux

Geffroy

et al. 2010

Clinical Cancer Research

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Purpose: Osteosarcoma and Ewing's sarcoma are high-grade neoplasms typically arising in the bones of children and adolescents. Despite improvement in therapy, the five-year survival rate is only 20% for patients not responding to treatment or presenting with metastases. Among new therapeutic strategies, the efficacy of tumor necrosis factor (TNF)-related apoptosis-inducing ligand (TRAIL), a member of the TNF superfamily with strong antitumoral activity and minimal toxicity to most normal cells and tissues, was investigated by complementary approaches both in vitro and in preclinical models.Experimental design: The sensitivity of osteosarcoma and Ewing's sarcoma cell lines to TRAIL was investigated in vitro by determining TRAIL receptor expression together with TRAIL effects on cell viability and apoptosis. Complementary preclinical studies were carried out in respective tumor models by inoculation of osteosarcoma or Ewing's sarcoma tumor cells in paraosseous location. In addition, a model of lung nodule dissemination was developed by i.v. injection of osteosarcoma cells.Results: In vitro, both osteosarcoma and Ewing's sarcoma cells that express the TRAIL death receptors were highly sensitive to TRAIL-induced caspase-8-mediated apoptosis. TRAIL administered in vivo by nonviral gene therapy inhibited primary bone tumor incidence and growth by 87% and prevented tumorinduced osteolysis, leading to a significant 2-fold increase in animal survival 40 days after tumor induction. Furthermore, TRAIL inhibited tumor nodule dissemination in lungs and increased survival in an osteosarcoma model.Conclusion: These findings suggest that TRAIL is a promising candidate for the development of new therapeutic strategies in the most frequent malignant primary bone tumors.

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Deskriptive Epidemiologie der Ewing-Tumoren - Analysen der deutschen Patienten von (EI) CESS 1980-1997*

Abstract: The large (EI)CESS database enables the meaningful and reliable description of epidemiological characteristics of the rare occurrence of the Ewing family of tumours. Further analyses of this database seem to hold great promise.

Cited by 45 publications

References 2 publications

Prognosis and Outcome of Adult Patients with Ewing Sarcoma (Local Experience)

Prognosis and Outcome of Adult Patients with Ewing Sarcoma (Local Experience)

EWS–ETS oncoproteins: The linchpins of Ewing tumors

Preclinical Evidence that Use of TRAIL in Ewing's Sarcoma and Osteosarcoma Therapy Inhibits Tumor Growth, Prevents Osteolysis, and Increases Animal Survival

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