No benefit of ifosfamide in Ewing's sarcoma: a nonrandomized study of the French Society of Pediatric Oncology.

1992 ◽  
Vol 10 (9) ◽  
pp. 1407-1412 ◽  
Author(s):  
O Oberlin ◽  
J L Habrand ◽  
J M Zucker ◽  
M Brunat-Mentigny ◽  
M J Terrier-Lacombe ◽  
...  
Keyword(s):  
Pediatric Oncology ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Disease Free Survival ◽  
Drug Regimen ◽  
French Society ◽  
Free Survival ◽  
Treatment Regimens ◽  
Chemotherapeutic Treatment ◽  
Disease Free

PURPOSE To undertake a new protocol with the goals of improving the chemotherapeutic treatment of pediatric Ewing's sarcoma by introducing ifosfamide, and to widen the indications for surgical resection of Ewing's tumor to obtain better local control and to reduce radiation doses. PATIENTS AND METHODS The French Society of Pediatric Oncology initiated its first cooperative Ewing's sarcoma study in 1978, using a four-drug regimen (cyclophosphamide, dactinomycin, Adriamycin [doxorubicin; Farmitalia Carlo Erba, Rueil-Malmaison, France], and vincristine). Ninety-five patients were included, and, at 5 years, the disease-free survival reached a plateau of 51%. After encouraging responses of recurrent soft tissue or bone sarcomas to ifosfamide, a second study began in 1984 using a new chemotherapy regimen in which cyclophosphamide was replaced by ifosfamide. Sixty-five patients were treated. RESULTS By February 1992, the median follow-up was 5.8 years. The estimated 5-year disease-free survival was 52%. We observed unexpected cardiac toxicity. Three patients experienced acute cardiac failure that was lethal in two cases. The acute toxicity of ifosfamide prompted us to stop the protocol. Retrospectively, the lack of efficacy reinforced our decision. CONCLUSION We conclude that ifosfamide did not improve the outcome of the patients despite the fact that these two treatment regimens were not randomized.

scholarly journals Primary Adult Renal Ewing's Sarcoma: A Rare Entity

Sarcoma ◽  
2009 ◽  
Vol 2009 ◽  
pp. 1-5 ◽  
Author(s):  
Ravindra Mukkunda ◽  
Ramachandran Venkitaraman ◽  
Khin Thway ◽  
Toon Min ◽  
Cyril Fisher ◽  
...  
Keyword(s):  
Adjuvant Chemotherapy ◽  
Metastatic Disease ◽  
Radical Nephrectomy ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Disease Free Survival ◽  
Adult Patients ◽  
Free Survival ◽  
Disease Free ◽  
Renal Origin

Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin.Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported.Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/−radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months.Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival.

Therapy for localized Ewing's sarcoma of bone.

1989 ◽  
Vol 7 (2) ◽  
pp. 208-213 ◽  
Author(s):  
F A Hayes ◽  
E I Thompson ◽  
W H Meyer ◽  
L Kun ◽  
D Parham ◽  
...  
Keyword(s):  
Induction Chemotherapy ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Disease Free Survival ◽  
Initial Response ◽  
Primary Tumors ◽  
Free Survival ◽  
Kaplan Meier ◽  
Wbc Count ◽  
Disease Free

Fifty-two previously untreated patients with localized Ewing's sarcoma of bone were treated with nonintensive chemotherapy in combination with surgery or radiation therapy (RT). RT was delivered to limited volumes in a dose dependent on the initial response to induction chemotherapy (30 to 35 Gy v 50 to 55 Gy). Fifty of the 52 patients achieved complete or partial responses with induction chemotherapy, with one nonresponding patient rendered free of tumor with surgery. Fifty patients were evaluable for local control of tumor and overall response to protocol therapy. Seventeen relapses have occurred; three metastatic, four local plus metastatic, and ten local. Two factors predicted worse disease-free survival: high WBC count (P = .03) and size of primary tumor (P = .05). Of the 14 local recurrences, 12 occurred in 28 patients who presented with primary tumors greater than 8 cm in size while only two of 22 patients with lesions less than 8 cm had local recurrence. The Kaplan-Meier estimate of disease-free survival at 3 years is 82% for those with small lesions and 64% for those with larger lesions. Site of primary was of no prognostic value (P = .27). The 5-year survival estimate for all patients is 80% (median time on study, 3.3 years).

scholarly journals Functional and Oncological Outcomes of Multidisciplinary Management of Ewing’s Sarcoma of Clavicle: A Single-Center Experience

2021 ◽  
Author(s):  
Ashish Gulia ◽  
Ajay Puri ◽  
Srinath Gupta ◽  
Tushar Vora ◽  
Siddhartha Laskar
Keyword(s):  
Functional Outcomes ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Disease Free Survival ◽  
Functional Evaluation ◽  
Musculoskeletal Tumor Society ◽  
Free Survival ◽  
Oncological Outcomes ◽  
Disease Free

Abstract Objectives Ewing’s sarcoma is best treated using a multidisciplinary approach. We discuss the functional and oncological outcomes of clavicular Ewing’s sarcoma which has been sparsely reported in literature. Materials and Methods We retrospectively evaluated patients who underwent resections for Ewing’s sarcoma of clavicle from January 2002 to December 2017. The study end points were locoregional recurrence free survival (LRFS), disease-free survival (DFS), and overall survival (OS) at 3 and 5 years, and functional outcomes measured by Musculoskeletal Tumor Society (MSTS) scores. Statistical Analysis The LRFS, DFS, and OS were calculated and analyzed using the Kaplan–Meier method and log-rank test. Results Data of 21 patients (male: 12, female: 9) was analyzed with a mean age [range] of 15.3 [6–40] years. Total clavicle excision was done in 62% (13 of 21) while 38% (n = 8) had partial resections. Radiotherapy was administered in 15 patients (71.5%). At a median follow-up of 42 months (range: 7–198), data of 20 patients was available for follow-up. Ten patients died (due to disease: nine, other reasons: One), eight are disease free and alive, one had metastasectomy on recurrence and is disease free and, one patient is alive with disease and on palliative chemotherapy. The LRFS, DFS, and OS were 95, 59, 65% and 95, 47, 59% at 3 and 5 years, respectively. The functional evaluation done for nine patients who are free of disease showed a mean MSTS score of 29 (range: 27–30; total clavicle excision: 28.5, partial clavicle excision: 29.5). Conclusion Patients with Ewing’s sarcoma of the clavicle who underwent resection without reconstruction have acceptable local control rates and excellent functional outcomes.

Ten Years Experience with Ewing's Sarcoma

1977 ◽  
Vol 63 (1) ◽  
pp. 77-89 ◽  
Author(s):  
Marco Gasparini ◽  
Sandro Barni ◽  
Angelo Lattuada ◽  
Renato Musumeci ◽  
Gianni Bonadonna ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Disease Free Survival ◽  
Local Therapy ◽  
New Combination ◽  
Multiple Drug ◽  
Free Survival ◽  
Median Disease Free Survival ◽  
Advanced Tumor ◽  
Disease Free

The series comprises 57 consecutive patients with Ewing's sarcoma admitted to the National Cancer Institute of Milan from 1965 to 1976. In 75 % the disease was confined to one bone, while in 25 % multiple bone and/or visceral lesions were present. Patients with clinically localized tumor treated before 1971 with local therapy, showed a median disease-free survival of 5 months. After 1971, radiotherapy and/or surgery to local tumor was combined with multiple drug chemotherapy (ADM, VCR, CTX) and the projected median disease-free survival increased to 24+ months. In previously untreated patients with advanced tumor adriamycin, used as single drug, achieved an overall response rate of 73 %. This is comparable to that achieved by a new combination including ADM, VCR, CTX, CCNU (75%). This multiple drug regimen is, however, expected to prolong the duration of response.

scholarly journals Nodular mixed lymphoma: results of a randomized trial failing to confirm prolonged disease-free survival with COPP chemotherapy

Blood ◽  
1981 ◽  
Vol 58 (5) ◽  
pp. 920-925 ◽  
Author(s):  
JH Glick ◽  
JM Barnes ◽  
EZ Ezdinli ◽  
CW Berard ◽  
EL Orlow ◽  
...  
Keyword(s):  
Randomized Trial ◽  
Eastern Cooperative Oncology Group ◽  
Disease Free Survival ◽  
Response Duration ◽  
Complete Response ◽  
Drug Regimen ◽  
Late Relapse ◽  
Hematologic Toxicity ◽  
Free Survival ◽  
Disease Free

Abstract Fifty-two patients with stage III or IV nodular mixed lymphocytic- histiocytic lymphoma (NM) were entered on a prospective randomized trial comparing cyclophosphamide-prednisone (CP) to either COPP (cyclophosphamide, vincristine, procarbazine, prednisone) or BCVP (BCNU, cyclophosphamide, vincristine, prednisone). The COPP regimen utilized in this Eastern Cooperative Oncology Group (ECOG) trial was similar to the four-drug regimen C-MOPP reported by the National Cancer Institute to achieve prolonged relapse-free survival in this histology. No significant differences in complete response rates, response duration, or overall survival were noted among the three regimens. A pattern of continuous late relapse was observed for all three chemotherapy programs. Although 11 of the 18 (61%) COPP patients achieved a complete response, only 3/11 (27%) remain disease-free with a median follow-up of over 3 yr. However, two of these three long-term complete responders have died with no clinical evidence of recurrent disease. The COPP patients received 84% of the calculated ideal doses of cyclophosphamide and 78% of the ideal dosage of procarbazine. Grade 3–4 hematologic toxicity was noted in 22% of the COPP group, 36% with BCVP, and 0% for the CP patients. We were unable to confirm the ability of COPP to achieve durable complete remissions in NM lymphoma. The cyclophosphamide-prednisone combination was equally effective when compared with COPP and BCVP, but produced minimal toxicity.

1198 Treatment of localized Ewing's sarcoma in young adults. A study of the French society of pediatric oncology

1995 ◽  
Vol 31 ◽  
pp. S250
Author(s):  
O. Oberlin ◽  
N.B. Bui ◽  
N. Dohollou ◽  
M.C. Demaille ◽  
M. Brunat-Mentigny ◽  
...  
Keyword(s):  
Young Adults ◽  
Pediatric Oncology ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
French Society

Non Hodgkin Lymphoma In Children From Northern Egypt

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5062-5062 ◽  
Author(s):  
Mohammed El Shazly ◽  
Waleed Arafaat ◽  
Ashraf el Masry ◽  
Aly Fahmy ◽  
Leslie E. Lehmann
Keyword(s):  
Pediatric Oncology ◽  
Abdominal Mass ◽  
Disease Free Survival ◽  
Stage Iv ◽  
Historical Cohort ◽  
Free Survival ◽  
Oncology Service ◽  
Northern Egypt ◽  
Pediatric Service ◽  
Disease Free

Abstract Data regarding the presentation and outcome of pediatric malignancies in northern Egypt, a resource-limited area, is limited. Alexandria School of Medicine (ASM) is the only center in Northern Egypt providing therapy to children with oncologic conditions. It serves 4 governorates and a population of 20 million. Initially children were treated as part of a combined adult/pediatric oncology service with therapy based on active adult protocols. In January 2010 a dedicated pediatric oncology service was created and staffed with providers specifically trained to care for children;international pediatric protcols were employed. We here present a comparison of outcomes achieved in children diagnosed with non-Hodgkin's lymphoma (NHL) before and after launching this service. Methods A retrospective review of patients <20 years old with NHL treated at ASM was performed. Those treated from 1/1999 to 12/2009 were the historical cohort. This group was compared to patients treated between 1/2010-12/ 2012 on the pediatric service. Results There were a total of 92 cases diagnosed with NHL in the historical cohort. This represented 23% of the total pediatric non-leukemic malignancies seen in this time at ASM. 38 cases of NHLwere diagnosed after the pediatric service opened representing 6% of the pediatric non-leukemic malignancies. In the historical group the most common presentation was abdominal mass (35%) followed by mediastinal lymphadenopathy (26%) , head and neck adenopathy (13%), pancytopenia (20%) , CNS involvement (10%) and other manifestations (10%). After the launch the most common presentation was abdominal mass (63%) followed by mediastinal adenopathy (18%) , pancytopenia ( 18%), head and neck adenopathy (15%) and other manifestations (8 %). ( Totals are >100% as some children had > one symptom at presentation.) Staging was done according to St. Jude classification of NHL. Historically the majority of patients presented with stage IV disease ( 51%). 25% were Stage III, 19% stage II and 5% stage I. After the launch the most common stage at presentation was stage II ( 42%) followed by stage III (40%) , stage IV ( 16%) and stage I ( 3%). Diagnosis of lymphoma was pathologically proven in 80% and 20% were treated based on clinical/radiologic signs and symptoms. Histopathological diagnosis in the older cohort were Burkitt lymphoma (39%), Lymphoblastic Lymphoma (30%), Diffuse large B cell lymphoma (DLBCL) (20%) and anaplastic lymphoma (ALCL) (11%). After the launch diagnoses were Burkitt lymphoma (63%), Lymphoblastic Lymphoma (15%), DLBCL (13%), ALCL (5%) and Natural Killer Cell lymphoma (4%). In the historical group pediatric patients were treated on adult protocols in use at that time. 60% received only chemotherapy 35% were treated with a combination chemotherapy/radiotherapy and 5% were treated with radiotherapy as the sole modality. After the launch patients were treated on internationally published pediatric protocols employing multiagent chemotherapy. No patients in this group received radiation therapy. With a follow up period of 24-132 (mean 49) months, 25% of historical patients succumbed to disease or therapy related complications for a disease free survival of 75%. After the establishment of a pediatric service with a follow up of 3-30 (mean 12) months 13% had died for a disease free survival of 87% ( p= 0.17). Conclusion NHL is one of the most common pediatric oncologic diagnoses and patients in developed countries have very good outcomes with > 80% survival on current protcols. At ASM, where the majority of children residing in northern Egypt receive their oncology care, children were treated as part of an adult oncology service, a common occurrence when resources are limited. 3 years ago the need for a dedicated pediatric service was recognized. We here compare outcomes for children with NHL before and after the establishment of this service. The subtypes seen are similar to those in Northern America and Europe and do not differ between the 2 periods. However disease free survival significantly improved in the recent era and is now similar to international norms. These results indicate that despite limitations encountered in this setting developing a dedicated pediatric oncology program can postivilet impact outcomes in addition to creating a platform for prospective data collection and practice improvement that will continue to improve results for these unfortunate children. Disclosures: No relevant conflicts of interest to declare.

High-dose melphalan, etoposide, and carboplatin followed by autologous stem-cell rescue in pediatric high-risk recurrent Wilms' tumor: a French Society of Pediatric Oncology study.

1998 ◽  
Vol 16 (10) ◽  
pp. 3295-3301 ◽  
Author(s):  
F Pein ◽  
J Michon ◽  
D Valteau-Couanet ◽  
E Quintana ◽  
D Frappaz ◽  
...  
Keyword(s):  
Stem Cell ◽  
High Risk ◽  
Pediatric Oncology ◽  
Wilms Tumor ◽  
Disease Free Survival ◽  
Stage Iv ◽  
High Dose ◽  
French Society ◽  
Time Curve ◽  
Disease Free

PURPOSE The three-drug combination of melphalan (M), etoposide (E), and carboplatin (C) followed by autologous stem-cell (ASC) rescue has been evaluated prospectively by the French Society of Pediatric Oncology (SFOP) in pediatric high-risk recurrent (HRR) Wilms' tumor (WT) patients with chemotherapy-responsive disease. PATIENTS AND METHODS From October 1988 to October 1994, 29 patients with HRR WT were treated in nine SFOP centers. Two additional patients with stage IV anaplastic WT were consolidated in first complete response (CR) with the same regimen and have been studied separately. The regimen consisted of M 180 mg/m2 for 1 day, E 200 mg/m2/d for 5 days, and C at a daily targeted area under the concentration-time curve (AUC) of 4 mg x min/mL for 5 days. ASCs were reinfused 48 hours after M. RESULTS Twelve of 28 assessable patients with HRR WT are still in continuous CR at a median of 48.5 months (range, 36 to 96) after consolidation. Disease-free survival (DFS) and overall survival (OS) estimated by the Kaplan-Meier method at 3 years were 50%+/-17% and 60%+/-18%, respectively. Sixteen patients relapsed at a median of 8.5 months (range, 3 to 53) after consolidation. Toxicity data are available in 31 grafted patients. Grade III and IV toxicities included hematologic side effects (n=31), hemorrhage (n=8), mucositis (n=24), diarrhea (n=12), renal disorders (n=8), and pneumonitis (n=3). CONCLUSION The adverse prognostic factors (APF) used to select patients for this dose-intensive chemotherapy define children with very-poor-risk recurrent WT. Despite high treatment-related toxicity, about half of these patients remain disease-free at 3 years. Patient outcome is statistically better when high-dose chemotherapy (HDCT) is performed as early as the second CR or partial response (PR). Novel therapeutic approaches with innovative preparative regimens are warranted for the remaining high-risk patients.

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