Observational Study on Skeletal Ewing's Sarcoma

Study Overview
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Brief Summary
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Observational prospective trial aimed to collect the collect demographic, clinical, surgical, pathological and molecular characteristics and treatment from patients affected by skeletal Ewing Sarcoma

Detailed Description
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The Ewing sarcoma treatment is based on chemotherapy, surgery and radiotherapy. Chemotherapy, performed with a combination of several drugs, is given as primary treatment before surgery. Surgical intervention must be performed in all cases in which it is possible to obtain an excision with free margins and with acceptable functional deficits. However, when, after surgery in case of incomplete resection, post-operative radiotherapy is used, in order to reduce the risk of local recurrence as much as possible. Subsequently in the local treatment, a phase of maintenance chemotherapy is foreseen, the intensity of which is modulated according to the response to induction therapy. The treatments are carried out with different programs for patients with localized disease or metastatic disease and derive from clinical trials carried out in the last 30 years. These results are the result of national and international prospective trials which, precisely due to the rarity of the disease, took many years to execute, and it is significant to know that randomized studies were in very limited numbers, as almost all the studies were represented by studies of uncontrolled phase II Improving treatments requires the expansion of knowledge on the biological behavior of this tumor and the acquisition of as much information as possible deriving from clinical experience. For this reason, pending the definition of a new prospective randomized trial, which will take time (approximately two years) to be made operational, it is considered useful from a scientific point of view to prospectively collect data relating to new cases of Ewing's sarcoma 'bone.

Official Title
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Observational Study on Treatment of Skeletal Ewing Sarcoma at Diagnosis

Conditions
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Ewing Sarcoma of Bone

Intervention / Treatment
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* Other: Treatment of Ewings Sarcoma of bone according clinical practice (includes drugs, surgery, radiotherapy or any other received treatments)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of Ewing's sarcoma of the bone Diagnosis of Ewing-like sarcoma (round cell sarcoma with fusion of the EWSR1 gene with others not of the ETS family, sarcoma with CIC rearrangement or sarcoma with BCOR rearrangement) Diagnosis of extraosseous Ewing's sarcoma for patients Age at diagnosis <50 years Assessment of the extent of the disease at onset in accordance with the European Society of Medical Oncology guidelines Patients or parents oe guardians of minors who have given their written informed consent to participate in the study Exclusion Criteria: 1. Presence of comorbid factors who can compromise the compliance of the treatment plan or the evaluation of the outcomes, including but not limited to organ pathologies that contraindicate the use of chemotherapy and psychological or social conditions that do not allow for adequate compliance treatment or adequate follow-up

Ages Eligible for Study
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Minimum Age: 0 Years
Maximum Age: 49 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Skeletal Ewing Sarcoma<br>This cohort include patients affected by Ewing Sarcoma of bone, referred to participating Institutions. | Other: Treatment of Ewings Sarcoma of bone according clinical practice (includes drugs, surgery, radiotherapy or any other received treatments)<br>* This observational study collects all the treatments received by the patients according clinical practices or experimental trials and therefore includes drug/biological/surgical/ radiotherapy and any other applicable treatments<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 5 years Event Free Survival (EFS) | Onset of any event (an event is the defined as disease recurrence, death for disease or any other cause) | 5 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Overall Survival (OS) | Time elapsed for the diagnosis to the death for any cause | at 5 years | 
| Adverse events related to the treatments in patients with age >21 years at the time of diagnosis | Number of Participants of age >21 years with Treatment-Related Adverse Events as Assessed by CTCAE v5.0, | Every 3 weeks (Week 3, week 6, week 9, week 12, ...) up to 30 months | 
| To assess baseline clinical and disease-specific factors with possible impact on survival analyses in the subpopulation with Ewing-like sarcoma | Collection of clinical pathological characteristics at disease presentation/diagnosis | Baseline | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Ewing Sarcoma