Italian Cooperative Study for the treatment of children and young adults with localized Ewing sarcoma of bone

Abstract

BACKGROUND In 1991, the Italian Association for Pediatric Hematology‐Oncology and the National Council of Research (CNR) initiated an Italian Cooperative Study (SE 91‐CNR Protocol) with the main objective of improving the overall survival (SUR) and the event free survival (EFS) of children and young adults with localized Ewing sarcoma and primitive neuroectodermal tumors of bone compared with a previous study (IOR/Ew2 Protocol). METHODS Between November 1991 and November 1997, 165 patients were enrolled in this study, 160 of whom were evaluable. The patients were treated with a multimodal approach characterized by intensified chemotherapy, hyperfractionated and accelerated radiation therapy, and the addition of ifosfamide and etoposide to standard chemotherapy with vincristine, actinomycin‐D, doxorubicin, and cyclophosphamide. RESULTS After a median follow‐up of 37 months, 126 of the 160 evaluable patients remained free of disease recurrence. Thirty‐one patients developed a disease recurrence (20 with disseminated disease). CONCLUSIONS The 3‐year SUR and EFS rates found in the current study (83.6% and 77.8%, respectively) may be considered satisfactory. Only age at diagnosis ≤14 years and a good histologic response appeared to affect the outcome of patients with localized Ewing sarcoma positively. These results appear to demonstrate the efficacy of the addition of ifosfamide in induction chemotherapy to four‐drug standard combination chemotherapy, as confirmed by the improved outcome in terms of 3‐year EFS reported in the SE 91‐CNR Protocol compared with the IOR/Ew2 Protocol (77.8% vs. 60.7%). In addition, the better outcome also could be explained by the change in treatment strategy with a trend toward the use of more surgery than radiation therapy compared with the authors' previous protocol. Cancer 1999;86:421–8. © 1999 American Cancer Society.

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.…”

“…[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). …”

“…Although some studies demonstrated no significant differences in the rates of local recurrence and/or survival between patients who received radiotherapy alone and patients who underwent surgery, 9,26,33 other studies demonstrated that surgery improved clinical outcomes significantly. 34,35 Results from 1058 patients who were treated on Cooperative Ewing Sarcoma Study 81 (CESS 81), CESS 86, and European Intergroup Cooperative Ewing Sarcoma Study 92 demonstrated that local control and EFS among patients who received definitive radiotherapy was significantly lower than EFS among patients who underwent surgery with or without receiving additional radiotherapy, although the former subgroup of patients represented a negatively selected population with unfavorable tumor sites.…”

“…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.…”

Sarcoma de Ewing: epidemiologia e prognóstico dos pacientes tratados no Instituto de Oncologia Pediátrica, IOP-GRAACC-UNIFESP

“…Our patients' ages were concentrated between four and 30 years. Age is a prognostic factor in the literature (16,17) , and this was observed in our sample, with lower five-year survival among patients over the age of 12 years (50.2%), compared with patients under the age of 12 years (71.7%) (P = 0.075). The mean age of the patients with pulmonary metastasis was slightly lower than the mean age among patients with extrapulmonary metastasis (respectively, P = 0.501 and P = 0.261), thus contrasting with local recurrence, which occurred in slightly older patients (P = 0.592).…”