Multidisciplinary treatment of primary Ewing's sarcoma of bone.A 6-year experience of a European cooperative trial

Abstract

The German Society of Pediatric Oncology in 1981 initiated the Cooperative Ewing's Sarcoma Study (CESS 81) using a four-drug combination of chemotherapy prior to definitive local control with surgery and/or radiation. From January 1, 1981 until February 28, 1985, 93 patients were registered at the trial office from 54 participating institutions in West Germany, Austria, Switzerland, and the Netherlands. On February 1, 1987, 54 of 93 patients were disease-free. Using the Kaplan-Meier life table analysis, the estimated disease-free survival (DFS) rate was 60% at 36 months and 55% at 69 months. The median period of observation was 29 months, ranging from 22 months to 69 months. Twenty-one of 93 patients (23%) had local failure, 18 of 93 patients (19%) developed systemic metastases. The local failure rate was particularly high in patients treated with radiation and was reduced when radiation planning was centralized within the study based upon the extent of disease at diagnosis. Cox regression analysis of prognostic factors showed that tumor volume was a significant factor influencing prognosis. The estimated 3-year DFS rate was 80% for patients with small tumors (volume less than 100 ml) compared to 31% for patients with large tumors (volume greater than or equal to 100 ml). In patients who had surgery for local control, the histologic response to chemotherapy was analyzed on the surgical specimen and had a strong influence on survival: 79% DFS at 3 years for patients with less than 10% viable tumor (good responders) compared to 31% DFS for patients with more than 10% viable tumor (poor responders). Tumor load and responsiveness to chemotherapy are the two major factors influencing prognosis in patients with primary Ewing's sarcoma of bone.

Intraoperative radiotherapy in the multidisciplinary treatment of bone sarcomas in children and adolescents

“…Through the combination of high-dose chemo-and radiotherapy is possible to achieve up to 70% local control in Ewing's sarcoma of the extremities [5,6]. These figures are apparently improved by adding surgery to the treatment management, although it might be compromised in tissues which have received high irradiation doses (over 50 Gy) [26].…”

“…Contemporary survival rates in patients with localized disease have been reported in the range of 65-75% for Ewing's sarcoma [4][5][6][7] and 65-90% for osteosarcoma [8,9].…”

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

“…The regimens that were applied are listed in Table 1. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] High-dose chemotherapy (HDC) with hematopoietic stem cell rescue was received by 51 patients, including 48 patients who received autologous peripheral blood stem cell transfusion (PBSCT) and 3 patients who underwent autologous bone marrow transplantation.…”

Prosthetic replacement surgery for bone tumours – cure at less cost?

“…In CESS 81 surgery for local control was carried out in 60 (65%) of 93 patients, 14 (15%) of these being extracompartmental resections rather than amputation (Jurgens et al, 1988). In CESS 86 74% of patients had primary surgery, 67% of these having resection rather than amputation (H. Jurgens, personal communication).…”

“…Even in patients treated on a common protocol in the US CCSG studies, no difference in survival was seen with the different types of surgery (Makley & Krailo, 1988). However the results of a multi-institutional study in Japan (Tomita & Tsuchiya, 1989) and in Europe (Jurgens et al, 1988) following surgery the number of patients rated 'excellent' or 'good' was high but the number rated as 'fair' or 'poor' increased after a 4 year follow up mainly due to post surgical complications (Tomita & Tsuchiya, 1989). Van der Eyken (personal communication) carried out a study 'in the field' of emotional adjustment and functional ability in a group of 36 adolescents who had either endoprosthetic replacement, amputation or rotationplasty and who were taking part in a skiing holiday.…”