Observational Study on Skeletal Ewing's Sarcoma

Официальное название

Observational Study on Treatment of Skeletal Ewing Sarcoma at Diagnosis

Обзор

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

Подробное описание

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.

Вмешательства

• Другое Treatment of Ewings Sarcoma of bone according clinical practice (includes drugs, surgery, radiotherapy or any other received treatments)
  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

Первичные конечные точки

• 5 years Event Free Survival (EFS) [Срок оценки: 5 years]

Вторичные конечные точки (3)

• Overall Survival (OS) [Срок оценки: at 5 years]
• Adverse events related to the treatments in patients with age >21 years at the time of diagnosis [Срок оценки: 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 [Срок оценки: Baseline]

Критерии участия

Критерии включения

• 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

Критерии исключения

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

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Да

Дизайн исследования

Модель наблюдения

Когортное

Центры проведения

Италия · 19 центров

• Istituto Clinico Humanitas — Rozzano
• Centro di Riferimento Oncologico - Unit of Medical Oncology — Aviano
• I.R.C.C. - Unit of Medical Oncology — Candiolo
• Azienda ospedaliero universitaria consorziale policlinico - bari — Bari
• A.O. Universitaria Policlinico S. Orsola Malpighi di Bologna — Bologna
• Istituto ortopedico Rizzoli — Bologna
• A.O. Universitaria Meyer — Florence
• Istituto Giannina Gaslini — Genova
• … и ещё 11 центров

Публикации

• Riggi N, Suva ML, Stamenkovic I. Ewing's Sarcoma. N Engl J Med. 2021 Jan 14;384(2):154-164. doi: 10.1056/NEJMra2028910. No abstract available. PMID 33497548
• Casali PG, Bielack S, Abecassis N, Aro HT, Bauer S, Biagini R, Bonvalot S, Boukovinas I, Bovee JVMG, Brennan B, Brodowicz T, Broto JM, Brugieres L, Buonadonna A, De Alava E, Dei Tos AP, Del Muro XG, Dileo P, Dhooge C, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Ferrari S, Frezza AM, Gaspar N, Gasperoni S, Gelderblom H, Gil T, Grignani G, Gronchi A, Haas RL, Hassan B, Hecker-Nolting S PMID 30285218
• Gaspar N, Hawkins DS, Dirksen U, Lewis IJ, Ferrari S, Le Deley MC, Kovar H, Grimer R, Whelan J, Claude L, Delattre O, Paulussen M, Picci P, Sundby Hall K, van den Berg H, Ladenstein R, Michon J, Hjorth L, Judson I, Luksch R, Bernstein ML, Marec-Berard P, Brennan B, Craft AW, Womer RB, Juergens H, Oberlin O. Ewing Sarcoma: Current Management and Future Approaches Through Collaboration. J Clin Oncol PMID 26304893
• Womer RB, West DC, Krailo MD, Dickman PS, Pawel BR, Grier HE, Marcus K, Sailer S, Healey JH, Dormans JP, Weiss AR. Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. J Clin Oncol. 2012 Nov 20;30(33):4148-54. doi: 10.1200/JCO.2011.41.5703. Epub 2012 Oct 22. PMID 23091096
• Ferrari S, Sundby Hall K, Luksch R, Tienghi A, Wiebe T, Fagioli F, Alvegard TA, Brach Del Prever A, Tamburini A, Alberghini M, Gandola L, Mercuri M, Capanna R, Mapelli S, Prete A, Carli M, Picci P, Barbieri E, Bacci G, Smeland S. Nonmetastatic Ewing family tumors: high-dose chemotherapy with stem cell rescue in poor responder patients. Results of the Italian Sarcoma Group/Scandinavian Sarcoma Grou PMID 21059639
• Luksch R, Tienghi A, Hall KS, Fagioli F, Picci P, Barbieri E, Gandola L, Eriksson M, Ruggieri P, Daolio P, Lindholm P, Prete A, Bisogno G, Tamburini A, Grignani G, Abate ME, Podda M, Smeland S, Ferrari S. Primary metastatic Ewing's family tumors: results of the Italian Sarcoma Group and Scandinavian Sarcoma Group ISG/SSG IV Study including myeloablative chemotherapy and total-lung irradiation. Ann PMID 22771824
• Dirksen U, Brennan B, Le Deley MC, Cozic N, van den Berg H, Bhadri V, Brichard B, Claude L, Craft A, Amler S, Gaspar N, Gelderblom H, Goldsby R, Gorlick R, Grier HE, Guinbretiere JM, Hauser P, Hjorth L, Janeway K, Juergens H, Judson I, Krailo M, Kruseova J, Kuehne T, Ladenstein R, Lervat C, Lessnick SL, Lewis I, Linassier C, Marec-Berard P, Marina N, Morland B, Pacquement H, Paulussen M, Randall R PMID 31553693
• Ladenstein R, Potschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, van den Berg H, Dirksen U, Hjorth L, Michon J, Lewis I, Craft A, Jurgens H. Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol. 2010 Jul 10;28(20):3284-91. doi: 10.1200/JCO.2009.22.9864. Epub 2010 Jun 14. PMID 20547982

Идентификаторы

NCT: NCT04845893 · ISG-EWOss

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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