Observational Study on Skeletal Ewing's Sarcoma (EWOss)

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

Study Overview

• Status: Recruiting
• Conditions: Ewing Sarcoma of Bone
• Intervention / Treatment: Other: Treatment of Ewings Sarcoma of bone according clinical practice (includes drugs, surgery, radiotherapy or any other received treatments)

Detailed Description

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.

• Study Type: Observational
• Enrollment (Estimated): 100

Contacts and Locations

Study Locations

• Italy
  • Bari, Italy, 70124
    • Recruiting
    • Azienda Ospedaliero Universitaria Consorziale Policlinico - Bari
    • Contact: Francesco De Leonardis, MD; Phone Number: 137 00390805592; Email: francesco.deleonardis@policlinico.ba.it
    • Principal Investigator: Francesco De Leonardis, MD
  • Bologna, Italy
    • Recruiting
    • Istituto Ortopedico Rizzoli
    • Contact: Toni Ibrahim, MD; Phone Number: ND; Email: toni.ibrahim@ior.it
  • Bologna, Italy, 40138
    • Recruiting
    • A.O. Universitaria Policlinico S. Orsola Malpighi di Bologna
    • Contact: Arcangelo Prete, MD; Phone Number: 111 +390516363; Email: tmoped@aosp.bo.it
    • Principal Investigator: Arcangelo Prete, MD
  • Firenze, Italy, 50139
    • Recruiting
    • A.O. Universitaria Meyer
    • Contact: Angela Tamburini, MD; Phone Number: 621 +3905556; Email: a.tamburini@meyer.it
    • Principal Investigator: Angela Tamburini, MD
  • Genova, Italy
    • Recruiting
    • Istituto Giannina Gaslini
    • Principal Investigator: Carla Manzitti, MD
    • Contact: Carla Manzitti, MD; Phone Number: ND; Email: carlamanzitti@gaslini.org
  • Milano, Italy
    • Recruiting
    • Fondazione IRCCS Istituto Nazionale dei Tumori
    • Contact: Roberto Luksch, MD; Phone Number: 2648 02.2390; Email: roberto.luksch@istitutotumori.mi.it
    • Principal Investigator: Roberto Luksch, MD
  • Napoli, Italy
    • Not yet recruiting
    • Azienda Ospedaliera Santobono Pausilipon
    • Contact: Massimo Eraldo Abate, MD; Phone Number: nd; Email: massimo.abate@gmail.com
  • Padova, Italy
    • Recruiting
    • Azienda Ospedaliera di Padova
    • Contact: Gianni Bisogno, MD; Phone Number: 461 039049-8211; Email: gianni.bisogno@unipd.it
    • Principal Investigator: Gianni Bisogno, MD
  • Palermo, Italy, 90127
    • Recruiting
    • ARNAS P. O. "Civico e Benfratelli"
    • Contact: Paolo D'Angelo, MD; Phone Number: 4316 039 091666; Email: oncoematologia.ped@arnascivico.it
  • Parma, Italy
    • Recruiting
    • Azienda Ospedaliera Universitaria di Parma
    • Contact: Patrizia Bertolini, MD; Phone Number: ND; Email: PBertolini@ao.pr.it
  • Pisa, Italy, 56126
    • Recruiting
    • Azienda Ospedaliero-Universitaria Pisana
    • Contact: Luca Coccoli, MD; Phone Number: 840 0039050992; Email: l.coccoli@ao-pisa.toscana.it
    • Principal Investigator: Luca Coccoli, MD
  • Roma, Italy
    • Not yet recruiting
    • Ospedale Pediatrico Bambin Gesu'
    • Contact: Giuseppe Maria Milano, MD; Phone Number: 111 06 68591; Email: giuseppemaria.milano@opbg.net
    • Principal Investigator: Giuseppe Maria Milano, MD
  • Rome, Italy, 00100
    • Recruiting
    • Istituto Regina Elena - IFO
    • Contact: Virginia Ferraresi, MD; Phone Number: +39065266919; Email: ferraresi@ifo.it
  • Torino, Italy, 10126
    • Recruiting
    • Ospedale Infantile Regina Margherita - Unit of Paediatric Oncoematology
    • Principal Investigator: Franca Fagioli, MD
    • Contact: Franca Fagioli, MD; Phone Number: 5447 +39011313; Email: franca.fagioli@unito.it
  • Trieste, Italy, 34137
    • Recruiting
    • IRCCS Materno Infantile Burlo Garofolo
    • Contact: Marco Rabusin, MD; Phone Number: 563 0403785; Email: rabusin@burlo.trieste.it
    • Principal Investigator: Marco Rabusin, MD
  • MI
    • Rozzano, MI, Italy, 20089
      • Recruiting
      • Istituto Clinico Humanitas
      • Contact: Alexia Bertuzzi, MD; Phone Number: +390282244540; Email: alexia.bertuzzi@cancercenter.humanitas.it
      • Principal Investigator: Alexia Bertuzzi, MD
  • Pordenone
    • Aviano, Pordenone, Italy, 33081
      • Recruiting
      • Centro di Riferimento Oncologico - Unit of Medical Oncology
      • Contact: Maurizio Mascarin, MD; Phone Number: 536 +390434659; Email: mascarin@cro.it
      • Principal Investigator: Maurizio Mascarin, MD
  • Torino
    • Candiolo, Torino, Italy, 10060
      • Recruiting
      • I.R.C.C. - Unit of Medical Oncology
      • Sub-Investigator: Sandra Aliberti, MD
      • Contact: Sandra Aliberti, MD; Email: sandra.aliberti@ircc.it

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 1 second to 49 years (Child, Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

Patients with Ewing's sarcoma of the bone or with Ewing-like sarcoma (round cell sarcoma with fusion of the EWSR1 gene with others not of the Ewings' Tumor Sarcoma family, sarcoma with Capicua Transcriptional Repressor (CIC) rearrangement or sarcoma with BCL6 corepressor (BCOR) rearrangement

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Skeletal Ewing Sarcoma; 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); 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

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: Italian Sarcoma Group

Publications and helpful links

General Publications

• 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.
• 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, Hohenberger P, Issels R, Joensuu H, Jones RL, Judson I, Jutte P, Kaal S, Kager L, Kasper B, Kopeckova K, Krakorova DA, Ladenstein R, Le Cesne A, Lugowska I, Merimsky O, Montemurro M, Morland B, Pantaleo MA, Piana R, Picci P, Piperno-Neumann S, Pousa AL, Reichardt P, Robinson MH, Rutkowski P, Safwat AA, Schoffski P, Sleijfer S, Stacchiotti S, Strauss SJ, Sundby Hall K, Unk M, Van Coevorden F, van der Graaf WTA, Whelan J, Wardelmann E, Zaikova O, Blay JY; ESMO Guidelines Committee, PaedCan and ERN EURACAN. Bone sarcomas: ESMO-PaedCan-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29(Suppl 4):iv79-iv95. doi: 10.1093/annonc/mdy310. No abstract available.
• 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. 2015 Sep 20;33(27):3036-46. doi: 10.1200/JCO.2014.59.5256. Epub 2015 Aug 24.
• 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. Erratum In: J Clin Oncol. 2015 Mar 1;33(7):814. Dosage error in article text.
• 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 Group III protocol. Ann Oncol. 2011 May;22(5):1221-1227. doi: 10.1093/annonc/mdq573. Epub 2010 Nov 8.
• 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 Oncol. 2012 Nov;23(11):2970-2976. doi: 10.1093/annonc/mds117. Epub 2012 Jul 5.
• 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 RL, Ranft A, Le Teuff G, Wheatley K, Whelan J, Womer R, Oberlin O, Hawkins DS; Euro-E.W.I.N.G. 99 and Ewing 2008 Investigators. High-Dose Chemotherapy Compared With Standard Chemotherapy and Lung Radiation in Ewing Sarcoma With Pulmonary Metastases: Results of the European Ewing Tumour Working Initiative of National Groups, 99 Trial and EWING 2008. J Clin Oncol. 2019 Dec 1;37(34):3192-3202. doi: 10.1200/JCO.19.00915. Epub 2019 Sep 25.
• 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.
• Whelan J, Le Deley MC, Dirksen U, Le Teuff G, Brennan B, Gaspar N, Hawkins DS, Amler S, Bauer S, Bielack S, Blay JY, Burdach S, Castex MP, Dilloo D, Eggert A, Gelderblom H, Gentet JC, Hartmann W, Hassenpflug WA, Hjorth L, Jimenez M, Klingebiel T, Kontny U, Kruseova J, Ladenstein R, Laurence V, Lervat C, Marec-Berard P, Marreaud S, Michon J, Morland B, Paulussen M, Ranft A, Reichardt P, van den Berg H, Wheatley K, Judson I, Lewis I, Craft A, Juergens H, Oberlin O; Euro-E.W.I.N.G.99 and EWING-2008 Investigators. High-Dose Chemotherapy and Blood Autologous Stem-Cell Rescue Compared With Standard Chemotherapy in Localized High-Risk Ewing Sarcoma: Results of Euro-E.W.I.N.G.99 and Ewing-2008. J Clin Oncol. 2018 Sep 6;36(31):JCO2018782516. doi: 10.1200/JCO.2018.78.2516. Online ahead of print.

Study record dates

Study Major Dates

• Study Start (Actual): June 1, 2021
• Primary Completion (Estimated): May 25, 2028
• Study Completion (Estimated): May 25, 2028

Study Registration Dates

• First Submitted: April 12, 2021
• First Submitted That Met QC Criteria: April 12, 2021
• First Posted (Actual): April 15, 2021

Study Record Updates

• Last Update Posted (Actual): September 13, 2023
• Last Update Submitted That Met QC Criteria: September 12, 2023
• Last Verified: September 1, 2023

More Information

Terms related to this study

• Keywords: Ewing Sarcoma
• Additional Relevant MeSH Terms: Neoplasms, Connective and Soft Tissue; Neoplasms by Histologic Type; Neoplasms; Osteosarcoma; Neoplasms, Bone Tissue; Neoplasms, Connective Tissue; Sarcoma; Sarcoma, Ewing
• Other Study ID Numbers: ISG-EWOss

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO
• IPD Plan Description: Not planned

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No