Primary Disseminated Multifocal Ewing Sarcoma: Results of the Euro-EWING 99 Trial

Abstract

PDMES patients may survive with intensive multimodal therapy. Age, tumor volume, and extent of metastatic spread are relevant risk factors. A score based on these factors may facilitate risk-adapted treatment approaches.

Tandem high-dose chemotherapy strategy as first-line treatment of primary disseminated multifocal Ewing sarcomas in children, adolescents and young adults

“…Prognostic factors (age, initial tumour volume, number of bone metastases, bone marrow and lung involvement) had allowed clinicians to define a prognostic score in this population. 6,7 The 3-year EFS rate was 50, 25 and 10% for a score of o 3, from 3 to 5 and for a score ⩾ 5, respectively. 6 The benefit of a myeloablative regimen in terms of outcome has been demonstrated in several tumour types with high sensitivity to HDC (for example, high-risk neuroblastomas).…”

“…6,7 The 3-year EFS rate was 50, 25 and 10% for a score of o 3, from 3 to 5 and for a score ⩾ 5, respectively. 6 The benefit of a myeloablative regimen in terms of outcome has been demonstrated in several tumour types with high sensitivity to HDC (for example, high-risk neuroblastomas). 8 In multimetastatic ES, the results of nonrandomised studies evaluating different tandem-HDC regimens remain controversial.…”

“…From October 2002 to 2009 (after closure of the EE99-R3 arm), patients younger than 25 years presenting with newly diagnosed metastatic ES, other than an isolated pulmonary metastasis (criterion for the EE99-R3 arm), 6 treated in the department of Oncology for child and adolescent at Gustave Roussy, were proposed a tandem-HDC strategy (HD-thiotepa followed by Mel/Bu) as local practice instead of the single-HDC Bu/Mel of the closed EE99-R3 arm trial. The study protocol was approved by the bone tumour committee of the SFCE (Société Française de lutte contre les Cancers et les leucémies de l'Enfant et de l'adolescent).…”

“…The PT volume was measured using the formula defined in the EE99 trial. 6 A chest CT (and/or abdomen and pelvis CT when appropriate) and whole-body technetium bone scintigraphy were used. Confirmatory X-rays and/or MRI for suspected metastases were recommended.…”

“…The 3-year EFS rate was 27% with the chemotherapy regimen in the R3 arm of the EuroEWING99 (EE99) trial. 6 This regimen comprised a six-course induction chemotherapy of vincristine/ifosfamide/doxorubicin/ etoposide (VIDE), then one course of vincristine/(d)actinomycin/ ifosfamide (VAI) and a single-HDC regimen by busulfan-melphalan (Bu/Mel), as consolidation therapy, followed by autologus peripheral haematopoietic progenitor (APHP) rescue. Prognostic factors (age, initial tumour volume, number of bone metastases, bone marrow and lung involvement) had allowed clinicians to define a prognostic score in this population.…”