Prognostic factors in localized Ewing's tumours and peripheral neuroectodermal tumours: the third study of the French Society of Paediatric Oncology (EW88 study)

Abstract

Purpose: (1) To improve survival rates in patients with Ewing's sarcoma (ES) or peripheral neuroectodermal tumours (PNET) using semi-continuous chemotherapy and aiming to peform surgery in all; (2) To identify early prognostic factors to tailor therapy for future studies. Patients and methods One hundred and forty-one patients were entered onto the trial between January 1988 and December 1991. Induction therapy consisted of five courses of Cytoxan, 150 mg/m2 × 7 days, followed by Doxorubicin, 35 mg/m2 i.v on day 8 given at short intervals. Surgery was recommended whenever possible. The delivery of radiation therapy was based on the quality of resection and the histological response to CT. Maintenance chemotherapy consisted of vincristine + actinomycin and cytoxan + doxorubicin. The total duration of therapy was 10 months. Results After a median follow-up of 8.5 years, the projected overall survival at 5 years was 66% and disease-free survival (DFS) was 58%. In patients treated by surgery, only the histological response to CT had an influence on survival: 75% DFS for patients with a good histological response (less than 5% of cells), 48% for intermediate responders and only 20% for poor responders (≥ 30% of cells), P < 0.0001. The initial tumor volume by itself had no influence on DFS in these patients. In contrast, the tumour volume had a strong impact on DFS in patients treated by radiation therapy alone. Age had no impact on outcome. Conclusion Therapeutic trials for localized Ewing's sarcoma should be based on the histological response to chemotherapy or on the tumour volume according to the modality used for local therapy. © 2001 Cancer Research Campaign http://www.bjcancer.com

Superficial primitive Ewing's sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases

“…7,11 Thanks to multimodal therapy associating initial chemotherapy and surgery with or without radiotherapy, the prognosis of ES/PNET has significantly improved, although it still remains relatively poor with a 5-year disease-free survival of about 60% for nonmetastatic patients at presentation. 14 According to the present study as well as two previously reported series, 7,8 superficial ES (ie, in the skin and/ or subcutis) has a better prognosis than deep-seated lesions. In our series, the patients who died from the tumor were treated by surgery only.…”

Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan

“…This result was comparable to the reports from other major series in ESFT of bone among Euro-American populations. 6,8,9,[23][24][25][26][27] Compared with the international variation in incidence, there was no considerable difference in the ratio of men to women, age distribution, or site distribution between Japanese patients and EuroAmerican patients (Table 2). Parkin et al also noted that there was no suggestion of any geographic or ethnic difference in the site distribution.…”

“…1 In the current study, the factors that were associated with decreased EFS were similar to those identified in previous retrospective studies. [27][28][29] It has been well confirmed that ESFT patients with larger tumors have a poorer outcome. 6,17,27,29,30 However, recent studies have demonstrated that this classic prognostic factor may become less critical when it is accompanied by the application of more aggressive treatment, such as EW-92, 25 SE 91-CNR, 9 and P6 8 (see Table 1).…”

Chemotherapy in Ewing’s sarcoma

“…Patients with metastatic disease confined to lung have a better prognosis than patients with extra pulmonary metastatic sites,12 while patients with metastasis to bone only seem to have a better outcome than patients with metastases to both bone and lung 7. Patients with minimal or no residual viable tumor after presurgical chemotherapy have a significantly better event-free survival compared with patients with larger amounts of viable tumor 89…”

“…The treatment plan generally consists of three stages: initial cytoreduction with chemotherapy to eradicate micro metastatic disease and facilitate effective local control measures with wide negative margins; definitive radiation or surgical therapy to eradicate all known disease; and consolidation therapy for eradication of occult residual disease to reduce the likelihood of tumor recurrence. Importantly, neoadjuvant chemotherapy not only helps achieve optimal cytoreduction to facilitate limb salvage procedures but also provides a chance to assess the response to chemotherapy 89…”