Extraskeletal Ewing's sarcoma family of tumors in adults: prognostic factors and clinical outcome.
Deniz Tural,Nil Molinas Mandel,Sergülen Dervişoğlu,Fazilet Oner Dincbas,Sedat Koca,Didem Çolpan Öksüz,Fatih Kantarci,Hande Turna,Fatih Selcukbiricik,Murat Hiz +9 more
TLDR
Adequate surgical resection, aggressive chemotherapy (vincristine, doxorubicin, cyclophosphamide and actinomycin-D alternating with ifosfamide and etoposide) and radiotherapy if indicated are the recommended therapy for patients with extraskeletal Ewing's sarcoma.Abstract:
Objective: The aim of this study was to evaluate prognostic factors, survival rate and the efficacy of the treatment modalities used in patients with extraskeletal Ewing’s sarcoma. Methods: Data of patients with extraskeletal Ewing’s sarcoma followed up at our center between 1997 and 2010 were retrospectively analyzed. Results: The median age of 27 patients was 24 years (range, 16‐54 years). The median follow-up was 31.8 months (range, 6‐144 months). Tumor size was between 1.5 and 14 cm (median: 8 cm). Eighty-five percent of patients had localized disease at presentation and 15% had metastatic disease. Local therapy was surgery alone in 16% of patients, surgery combined with radiotherapy in 42% and radiotherapy alone in 27%. All patients were treated with vincristine, doxorubicin, cyclophosphamide and actinomycin-D, alternating with ifosfamide and etoposide every 3 weeks. In patients with localized disease at presentation, the 5-year event-free survival and overall survival were 59.7 and 64.5%, respectively. At univariate analysis, patients with tumor size � 8 cm, high serum lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy and positive surgical margin had significantly worse event-free survival. The significant predictors of worse overall survival at univariate analysis were tumor size 8� cm, high lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin. Conclusions: Prognostic factors were similar to primary osseous Ewing’s sarcomas. Adequate surgical resection, aggressive chemotherapy (vincristine, doxorubicin, cyclophosphamide and actinomycin-D alternating with ifosfamide and etoposide) and radiotherapy if indicated are the recommended therapy for patients with extraskeletal Ewing’s sarcoma.read more
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Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management
George Galyfos,Georgios Karantzikos,Nikolaos Kavouras,Argiri Sianou,Konstantinos Palogos,Konstantinos Filis +5 more
TL;DR: Pat characteristics and outcomes seem to be different in EES compared to patients with skeletal Ewing sarcoma, with implications for patient care and prognosis, and multimodality therapeutic strategies are recommended for all types of the Ewing tumour family.
Journal ArticleDOI
Evaluation of outcome and prognostic factors in extraosseous Ewing sarcoma
Bivas Biswas,N.K. Shukla,S.V.S. Deo,Sandeep Agarwala,Dayanand Sharma,Sreenivas Vishnubhatla,Sameer Bakhshi +6 more
TL;DR: Examination of extraosseous Ewing sarcoma with uniform chemotherapy protocol in patients aimed to examine this aspect in patients, identify prognostic factors and compare the same with osseously Ewing Sarcoma.
Journal ArticleDOI
International expert opinion on patient-tailored management of soft tissue sarcomas.
Jean-Yves Blay,Stefan Sleijfer,Patrick Schöffski,Akira Kawai,Thomas Brodowicz,George D. Demetri,Robert G. Maki +6 more
TL;DR: A model describing factors affecting treatment decisions in sarcoma was established and the model requires validation and several of its parameters require standardisation.
Journal ArticleDOI
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients.
Bhanusupriya Somarouthu,Atul B. Shinagare,Michael H. Rosenthal,Harika Tirumani,Jason L. Hornick,Nikhil H. Ramaiya,Sree Harsha Tirumani +6 more
TL;DR: Adult EESs are large tumours, which frequently invade adjacent organs and metastasize to the lung, which are larger, have more frequent metastases and poorer outcomes in adult extraskeletal Ewing sarcoma.
Journal ArticleDOI
Prognostic significance of serum lactate dehydrogenase levels in Ewing's sarcoma: A meta‑analysis
TL;DR: A meta-analysis demonstrates that high levels of serum LDH are associated with lower OS and 5-year DFS rates in patients with Ewing's sarcoma, and is an effective biomarker of Ewing’s Sarcoma prognosis.
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Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Holcombe E. Grier,Mark Krailo,Nancy J. Tarbell,Michael P. Link,Chris Fryer,Douglas J. Pritchard,Mark C. Gebhardt,Paul S. Dickman,Elizabeth J. Perlman,Paul A. Meyers,Sarah S. Donaldson,Sheila Moore,Aaron R. Rausen,Teresa J. Vietti,James S. Miser +14 more
TL;DR: The addition of ifosfamide and etoposide to a standard regimen does not affect the outcome for patients with metastatic disease, but it significantly improves the outcomeFor patients with nonmetastatic Ewing's Sarcoma, primitive neuroectodermal tumor of bone, or primitive sarcoma of bone.
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MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration.
Inge M. Ambros,Peter F. Ambros,Sabine Strehl,Heinrich Kovar,Helmut Gadner,Mechthild Salzer-Kuntschik +5 more
TL;DR: Both ES and pPNET cells express the MIC2 gene in very high amounts, which represents a highly selective and almost unique feature of these cells, making an assignment of these tumors in one entity even more likely.
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Extraskeletal neoplasm resembling Ewing's sarcoma
TL;DR: The pathologic features and the behavior of 39 small, round, or oval cell sarcomas occurring in the soft tissues and considered histologically indistinguishable from Ewing's sarcoma of bone are reviewed.