Pharmacological management of Ewing sarcoma family of tumours

Rodríguez‐Galindo, Carlos

doi:10.1517/14656566.5.6.1257

Cited by 11 publications

(8 citation statements)

References 62 publications

(69 reference statements)

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“…These five consecutive institutional studies recapitulate the incremental advances made internationally in the management of ESFT [11][12][13][14]. Treatments built on the efficacy of the four-drug regimen used in ES79 [5] by first adding the ifosfamide-etoposide (IE) combination (EW87) [6] and then intensifying therapy, adding granulocyte colony-stimulating factor, and improving support measures (EWI92, SJBCM and HIRISA) [2,15]. As systemic approaches evolved, so did local control.…”

Section: Discussionmentioning

confidence: 99%

Prognostic factors for local and distant control in Ewing sarcoma family of tumors

et al. 2008

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Results: The median age at diagnosis was 13.7 years. Ninety-five patients relapsed at a median of 1.6 years. The 5-year overall survival estimate was 63.5% 6 3.5%. The estimated 5-year cumulative incidence (CI) of local failure was 25.1% 6 3.0%. Local failure was associated with treatment era (P < 0.001), tumor size (P = 0.037) and type of local control (P = 0.021). Systemic treatment intensification improved local control. The estimated 5-year CI of distant recurrence was 22.5% 6 2.9%. Patients with localized disease (P < 0.001), smaller tumors (P = 0.018) and those who received surgery 6 radiation for local control (P = 0.023) had lower CI of distant failure.Conclusions: Successful treatment of ESFT requires optimal systemic and local therapy. Both treatment modalities are intertwined and the control of both local and distant disease is the result of the combined approach.

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Section: Discussionmentioning

confidence: 99%

Prognostic factors for local and distant control in Ewing sarcoma family of tumors

et al. 2008

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“…These two components are carried out in three phases: (1) induction chemotherapy, the goal of which is to achieve rapid initial cytoreduction and facilitate local control, (2) local control using surgery, radiation therapy, or both, usually after 10-12 weeks of induction chemotherapy, and (3) continuation therapy, which consists of chemotherapeutic regimens similar to those used for induction. 97 …”

Section: Therapy and Clinical Outcome Therapymentioning

confidence: 99%

“…6). 10,97 Furthermore, pelvic ESFT is commonly insidious and patients tend to present later in the disease course with advanced-stage disease. In one study, 25% of patients with primary pelvic ESFT had metastasis at diagnosis compared with 16% for patients with tumors arising at other sites.…”

Section: Outcome and Prognostic Factorsmentioning

confidence: 99%

Ewing Sarcoma Family of Tumors

Khoury

2005

Advances in Anatomic Pathology

167

127

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The Ewing sarcoma family of tumors (ESFT) comprises morphologically heterogeneous tumors that are characterized by nonrandom chromosomal translocations involving the EWS gene and one of several members of the ETS family of transcription factors. The translocation t(11;22)(q24;q12) is the most common and leads to the formation of the EWS-FLI1 fusion protein, which contributes to ESFT pathogenesis by modulating the expression of target genes. Tumors may be composed of small uniform cells with minimal morphologic evidence of differentiation, or they may be composed of larger, less uniform cells with varying degrees of neuroectodermal differentiation. CD99 expression is identified in nearly all ESFT and constitutes a useful positive marker when used as part of a panel of immunostains that can help rule out other differential diagnostic considerations. Molecular diagnostic tests commonly used to detect the presence of ESFT-specific translocations include RT-PCR and fluorescence in situ hybridization. Current therapy for patients with ESFT includes chemotherapy and surgery with or without radiation therapy. At present, the most significant prognostic factor for patients with ESFT is whether the disease is localized or metastatic.

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“…This dose intensification and interval compression has the theoretical advantage of allowing less time for recovery of the partially resistant cells. The efficacy of interval compression is currently being evaluated by the Children's Oncology Group (COG) AEWS-0031 study [16].…”

Section: Systemic Treatment: Chemotherapy Evolution Of Chemotherapeutmentioning

confidence: 99%

“…Treatment regimens using high doses of alkylating agents or topoisomerase II inhibitors (etoposide) have been associated with a high cumulative incidence of treatment-related acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS) [1,16]. The incidence in most studies has been reported to be approximately 8%, however up to a 22.7% incidence was noted in a small study of high-risk patients treated with very high-dose chemotherapy [17][18][19].…”

Section: Systemic Treatment: Chemotherapy Evolution Of Chemotherapeutmentioning

confidence: 99%

Current treatment for Ewing’s sarcoma

Thacker¹,

Temple²,

Scully³

2005

Expert Review of Anticancer Therapy

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Ewing's sarcoma is the second most common primary bone tumor seen in children and adolescents, and was described by James Ewing in 1921 as a diffuse endothelioma of bone. It is one of the differential diagnoses of pediatric small round blue cell tumors. This is not a single condition, but a group of morphologically and clinically closely related disorders with similar molecular biology -- expression of tumor-specific chimeric oncoproteins through balanced chromosomal translocations involving the EWS gene -- often referred to as the Ewing family of tumors. This includes Ewing's sarcoma of bone, extra-osseous Ewing's sarcoma, Askin tumor and peripheral neuroectodermal tumor. These are aggressive neoplasms with almost 25% of patients having clinically evident metastases at presentation. Ewing's sarcoma has therefore been considered as a systemic disease necessitating local as well as systemic treatment. An aggressive multidisciplinary approach has resulted in significant improvement in prognosis for patients with these tumors. Despite aggressive treatment, 20-40% of patients with localized disease and almost 80% of patients with metastatic disease at presentation succumb to the illness. Advances in understanding the molecular biology of these tumors will hopefully result in the development of novel treatment approaches. The aim of this article is to review the existing treatment methods and to highlight the more recent approaches to the treatment of this condition.

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Pharmacological management of Ewing sarcoma family of tumours

Cited by 11 publications

References 62 publications

Prognostic factors for local and distant control in Ewing sarcoma family of tumors

Prognostic factors for local and distant control in Ewing sarcoma family of tumors

Ewing Sarcoma Family of Tumors

Current treatment for Ewing’s sarcoma

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