The evidence in the literature supports the opinion that both non-cytotoxic and cytotoxic chemotherapies are effective against AF. However, the lack of sufficient patient numbers and randomized trials compromises the validity of the reported results and mandates further investigation with properly designed prospective studies including larger patient numbers, with main end points to include not only tumor response rate and survival but also quality-of-life issues.

No abstract

Summary: Patients and methodsEighteen patients were entered into the study. There were Eighteen patients with poor risk Ewing's sarcoma (including 11 patients with metastatic disease at nine females. The median age at diagnosis was 14.2 years (range 2.75-30 years). Primary tumours were of the central presentation) received consolidation therapy of busulphan and melphalan with autologous stem cell rescue.skeleton in 10 cases and long bones in eight cases. Eleven cases were metastatic at initial presentation, 10 with pulThere were nine females. The median age at diagnosis was 14.2 years (range 2.75-30 years). There was one monary disease and one with bone marrow involvement. The primary tumour of the metastatic cases was central early death due to cytomegalovirus pneumonitis. One patient developed a single generalised convulsion during skeleton in six, and long bones in five cases. Six of the seven non-metastatic patients had bulky primary tumours busulphan therapy. Severe renal toxicity was not encountered. One patient developed veno-occlusive dis-(у100 ml) and the seventh patient was in second complete remission at the time of high-dose therapy. In total 12 ease of the liver (VOD) which eventually resolved. With a median follow up of 2 years, 13 patients survive patients were in first complete remission (CR1), four in CR2, one in partial remission (PR) and one with progressincluding six with initial metastatic disease. We conclude that high-dose busulphan/melphalan is well-tolerive disease (PD) at the time of HDT.

Introduction: Adjuvant therapy in osteosarcoma (OS) and Ewing's sarcoma (ES) is primarily directed towards treatment of subclinical lung disease. Before the advent of modern intensive chemotherapy, lung irradiation was the only available adjuvant treatment. It has proven biological activity and low morbidity. There is, however, a wide variation in its application between centres. This systematic review aims to define the evidence to support the use of lung irradiation in these diseases.Design: A review of trials published between 1966 and 2000 was undertaken to determine the evidence for the use of pulmonary irradiation in OS and ES.Results: Several small series of prophylactic lung irradiation (PLI) have been reported, most from over 20 years ago. These studies support the theoretical basis for the use of PLI in both OS and ES. Few randomised studies have been performed which include PLI. In OS, studies demonstrated a trend in favour of PLI compared with no adjuvant treatment and, subsequently, a level of benefit similar to that achieved with chemotherapy, but no additive effect. No studies have used PLI in addition to current standard chemotherapy regimens, or evaluated its use after successful metastatectomy. In ES, only one randomised study has addressed the role of PLI, in a comparison with vincristine, actinomycin D and cyclophosphamide combination chemotherapy with or without doxorubicin. Prolonged follow-up favoured four-drug chemotherapy. Retrospective reports from large cooperative groups suggest that the addition of whole-lung radiotherapy (WLRT) improves outcome in ES patients presenting with pulmonary metastases. However, there are no randomised study data to support this. Conclusions:Further randomised studies are necessary to clarify the role of PLI in addition to current standard chemotherapy regimens, or its use after successful metastasectomy in patients with OS. In patients with localised ES adjuvant chemotherapy appears to be superior to PLI alone, while there is little evidence to support treatment with WLRT in patients who present with pulmonary metastases.

Curative therapies for Ewing sarcoma have been developed within cooperative groups. Consecutive clinical trials have systematically assessed the impact and timing of local therapy and the activity of cytotoxic drugs and their combinations. They have led to an increase of long-term disease-free survival to around 70% in patients with localized disease. Translational research in ES remains an area in which interdisciplinary and international cooperation is essential for future progress. This article reviews current state-of-the art therapy, with a focus on trials performed in Europe, and summarizes novel strategies to further advance both the cure rates and quality of survival.