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  • Florent Guérin,
  • Hélène Martelli,
  • Timothy Rogers,
  • Ilaria Zanetti,
  • Sheila Terwisscha van Scheltinga,
  • Federica De Corti,
  • Gabriela Guillén Burrieza,
  • Veronique Minard-Colin,
  • Daniel Orbach,
  • Max van Noesel,
  • Marie Karanian,
  • Raquel Davila Fajardo,
  • Johannes Merks,
  • Andrea Ferrari,
  • Gianni Bisogno
Florent Guérin
Assistance Publique - Hopitaux de Paris

Corresponding Author:florent.guerin@aphp.fr

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Hélène Martelli
Assistance Publique - Hopitaux de Paris
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Timothy Rogers
University Hospitals Bristol and Weston NHS Foundation Trust
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Ilaria Zanetti
AIL Padova
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Sheila Terwisscha van Scheltinga
Prinses Maxima Centrum voor Kinderoncologie
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Federica De Corti
Universita degli studi di Padova Dipartimento di Geoscienze
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Gabriela Guillén Burrieza
Hospital Universitari Vall d'Hebron Servei de Farmacologia Clinica
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Veronique Minard-Colin
Institut Gustave Roussy Departement de Medecine Oncologique
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Daniel Orbach
Universite Paris Sciences et Lettres Bibliotheques
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Max van Noesel
Prinses Maxima Centrum voor Kinderoncologie
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Marie Karanian
Centre Leon Berard Departement de Radiotherapie
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Raquel Davila Fajardo
The Ohio State University Department of Radiation Oncology
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Johannes Merks
Prinses Maxima Centrum voor Kinderoncologie
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Andrea Ferrari
Fondazione IRCCS Istituto Nazionale dei Tumori
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Gianni Bisogno
AIL Padova
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Background To assess the outcomes of pediatric patients with Undifferentiated Embryonal Sarcoma of the Liver (UESL) and treatment including at least surgery and systemic chemotherapy. Methods This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three european trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT) and chemotherapy. Results Out of 65 patients with a median age at diagnosis of 8.7 years (0.6-20.8), 15 had T2 tumors, and 1 had lymph node spread, 14 were Intergroup Rhabdomyosarcoma Study (IRS) I, 9 IRSII, 38 IRSIII, and 4 IRSIV. Twenty-eight upfront surgeries resulted in 5 operative spillages and 11 infiltrated surgical margins, whereas 37 delayed surgeries resulted in no spillages (P= 0.0119) and 3 infiltrated margins (P=0.0238). All patients received chemotherapy, including anthracyclines in 47. Radiotherapy was administered in 15 patients. With a median follow-up of 78.6 months, 5 year overall and event free survivals (EFS) were 90.1% (95%CI 79.2-95.5) and 89.1% (95%CI 78.4-94.6), respectively. Two out 4 local relapses had previous infiltrated margins and 2 out of 3 patients with metastatic relapses received reduced doses of alkylating agents. Infiltrated margins (P=0.1607), T2 stage (P=0.3870), use of RT (P= 0.8731), and anthracycline-based chemotherapy (P= 0.1181) were not correlated with EFS. Conclusions Neoadjuvant chemotherapy for pediatric patients with UESL increases the probability of complete surgical resection. The role of anthracyclines and radiotherapy for localized disease remains unclear. The use of alkylating agents is recommended.
06 Oct 2022Submission Checks Completed
06 Oct 2022Assigned to Editor
06 Oct 2022Submitted to Pediatric Blood & Cancer
12 Oct 2022Reviewer(s) Assigned
01 Nov 2022Review(s) Completed, Editorial Evaluation Pending
09 Nov 2022Editorial Decision: Revise Major
27 Feb 2023Submission Checks Completed
27 Feb 2023Assigned to Editor
27 Feb 20231st Revision Received
03 Mar 2023Review(s) Completed, Editorial Evaluation Pending
06 Mar 2023Reviewer(s) Assigned
20 Mar 2023Editorial Decision: Revise Minor