Observational Study on Skeletal Ewing's Sarcoma

Observational Study on Treatment of Skeletal Ewing Sarcoma at Diagnosis

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

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.

Inclusion Criteria:

1. Diagnosis of Ewing's sarcoma of the bone
2. 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)
3. Diagnosis of extraosseous Ewing's sarcoma for patients
4. Age at diagnosis <50 years
5. Assessment of the extent of the disease at onset in accordance with the European Society of Medical Oncology guidelines
6. 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