Localized Ewing Tumor of Bone: Final Results of the Cooperative Ewing’s Sarcoma Study CESS 86

Paulussen, Michael; Ahrens, S.; Dunst, Jürgen; Winkelmann, Winfried; Exner, G. U.; Kotz, R.; Amann, Gabriele; Dockhorn‐Dworniczak, Barbara; Harms, D.; Müller-Weihrich, St.; Welte, Karl; Kornhuber, B.; Janka-Schaub, G. E.; Göbel, U.; Treuner, J.; Voûte, P. A.; Zoubek, A.; Gadner, Helmut; Jürgens, Heribert

doi:10.1200/jco.2001.19.6.1818

Cited by 401 publications

(251 citation statements)

References 38 publications

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“…[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). …”

Section: Discussionmentioning

confidence: 99%

“…Although the histologic response to initial chemotherapy was among the most reliable predictive factors, 16,17,28,31,32 even with the limitation that tumors had to be resected to determine response, we also used simple radiographic evaluations based on changes in extraosseous tumor size. These evaluations also demonstrated sufficient predictive value.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Treatmentmentioning

confidence: 99%

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

Obata

Ueda

Kawai

et al. 2007

Cancer

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BACKGROUND.Ewing sarcoma family of tumors (ESFT) of bone is extremely rare in Japan. The objectives of the current study were to assess the clinical outcome and prognostic factors of patients with ESFT of bone in Japan and to compare them between Euro‐American and Japanese populations.METHODS.The authors conducted a retrospective analysis of 243 patients who were treated for ESFT of bone in Japan between 1981 and 2003. Local therapy was surgery in 35% of patients, surgery combined with radiotherapy in 40% of patients, radiotherapy alone in 22% of patients, and no local treatment in 3% of patients. All but 3 patients received various regimens of multidrug chemotherapy.RESULTS.The median patient age was 16 years. The primary disease sites were the trunk in 53% of patients and the extremities in 47% of patients. Forty‐one patients had metastases at presentation. The median follow‐up was 66 months. A univariate survival analysis demonstrated that patients who had metastases at presentation, primary site in the trunk, age ≥16 years, tumor size ≥10 cm, tumor that responded poorly to induction chemotherapy, and local treatment with radiotherapy alone had a significantly worse event‐free survival (EFS). A multivariate analysis further verified that the former 3 factors were significant adverse prognostic factors. Of 201 patients with localized disease, 45 patients who received current chemotherapy regimens that included ifosfamide and etoposide had a significantly better 5‐year EFS rate (67.6%) compared with other patients.CONCLUSIONS.The clinical outcome of patients with localized ESFT of bone in Japan has improved markedly with the use of current chemotherapy regimens that include ifosfamide and etoposide and has become comparable to the outcomes observed in other major series of Euro‐American patients. The prognostic factors are also almost identical. Cancer 2007 © 2007 American Cancer Society.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

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

Obata

Ueda

Kawai

et al. 2007

Cancer

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“…Factors involved in the prognosis include site of the tumor, tumor volume, age, sex, resection rate of tumor, presence of metastasis, treatment response, anemia, fever, and an increase in LDH [13][14][15]. For 20 years, the gold standard in the treatment of Ewing sarcoma of the limb is the combination of neo-adjuvant chemotherapy and enbloc resection.…”

Section: Discussionmentioning

confidence: 99%

Ewing sarcoma of the thoracic epidural space in a young child

et al. 2012

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Purpose To report on the clinical course and treatment of Ewing sarcoma of the thoracic epidural space in a 5-yearold girl. Methods We present the case of a 5-year-old girl who experienced back pain (day 1); on day 10, the pain had exacerbated and involuntary movements in the lower limbs occurred, and an MRI performed in her local hospital revealed a tumor lesion at the upper thoracic level. Results On day 13, emergency surgery was performed for partial resection of the tumor. Pathological examination of the resected tumor by immunostaining and gene testing revealed that it was MIC2 positive and an EWS-FLI 1 chimera, respectively, and Ewing sarcoma was diagnosed. The involuntary movements resolved immediately after the surgery. Three weeks after the operation, chemotherapy and radiation therapy were commenced. After 5 months, deep tendon reflexes recovered to normal. MRI showed that the tumor has not recurred at 29 months after surgery. Conclusions The majority of epidural patients undergo emergency surgery only after symptom exacerbation, which includes the development of neurological deficits. Thus, preoperative diagnosis of Ewing sarcoma of the epidural space is difficult and diagnosis is frequently made by a post-operative gene test. The resection area is limited to the intralesional margin area because a larger resection is difficult due to the characteristics of the affected region; thus, there is a higher possibility of recurrence and careful follow-up of the case is necessary.

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“…An EFS of 70% was achieved for patients with localized disease, and OS reached 80%. [4][5][6][7][8][17][18][19][20][21] The outcome of metastatic patients remains dismal, especially for patients with bone and BM involvement. [4][5][6][9][10][11] Rodriguez Galindo et al 23 suggested risk stratification, taking into account simple clinical features, such as age, stage, tumor volume, localization and histological response to chemotherapy, to modulate treatment intensification in different subgroups.…”

Section: Discussionmentioning

confidence: 99%

Toxicity of high-dose chemotherapy with etoposide, thiotepa and CY in treating poor-prognosis Ewing's sarcoma family tumors: the experience of the Bambino Gesù Children's Hospital

Ilari

Ioris

Milano³

et al. 2010

Bone Marrow Transplant

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We report the toxicity of high-dose chemotherapy (HDC) based on etoposide, thiotepa and CY (ETC) in children with poor-prognosis Ewing's sarcoma family tumors (ESFTs). A total of 26 patients with high-risk ESFT (metastasis or axis localization or tumor volume 4200 ml or necrosis o95%) were reviewed. The conditioning was based on etoposide (600 mg/m 2 ), thiotepa (750 mg/m 2 ) and CY (120 mg/kg) followed by autologous BM or PBSC rescue. The conditioning regimen was well tolerated, without any toxic deaths. The median time from transplant to a neutrophil count of 40.5 Â 10 9 /l was 10 days (range 6-27) and 22.5 days (range 9-114) for a plt count of 450 Â 10 9 /l. Oral mucositis was recorded in 20 patients, grade 1/2 in 19 and grade 3 in the last patient. Diarrhea grade 1/2 was recorded in four patients and grade 1/2 liver toxicity in four patients. Sepsis was documented in four cases and skin toxicity in three. Lung and tubular toxicity, respectively, were reported in one patient each. We conclude that the ETC regimen presented a limited and manageable toxicity. Further studies would confirm the role of ETC in high-risk ESFT.

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Localized Ewing Tumor of Bone: Final Results of the Cooperative Ewing’s Sarcoma Study CESS 86

Abstract: Fifty-two percent of CESS 86 patients survived after risk-adapted therapy. High-risk patients seem to have benefited from intensified treatment that incorporated ifosfamide.

Cited by 401 publications

References 38 publications

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

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

Ewing sarcoma of the thoracic epidural space in a young child

Toxicity of high-dose chemotherapy with etoposide, thiotepa and CY in treating poor-prognosis Ewing's sarcoma family tumors: the experience of the Bambino Gesù Children's Hospital

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