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ORIGINAL RESEARCH article

Front. Oncol.

Sec. Pediatric Oncology

Volume 15 - 2025 | doi: 10.3389/fonc.2025.1653015

This article is part of the Research TopicRecent Advances on PET-CT in Patients with Paediatric SarcomasView all articles

PAZOPANIB IN PATIENTS WITH PRIMARY MULTI-METASTATIC BONE

Provisionally accepted
Anna  RaciborskaAnna Raciborska1*Katarzyna  BilskaKatarzyna Bilska1Jadwiga  Węcławek-TompolJadwiga Węcławek-Tompol2Dorota  Sega-PondelDorota Sega-Pondel2Anna  ZelwiańskaAnna Zelwiańska1Borys  PrzybyszewskiBorys Przybyszewski1Radoslaw  ChaberRadoslaw Chaber3Renata  TomaszewskaRenata Tomaszewska4Justyna  Antoniuk-MajchrzakJustyna Antoniuk-Majchrzak1Tomasz  KozińskiTomasz Koziński1
  • 1Institute of Mother and Child, Warsaw, Poland
  • 2Uniwersytet Medyczny im Piastow Slaskich we Wroclawiu, Wrocław, Poland
  • 3Uniwersytet Rzeszowski, Rzeszow, Poland
  • 4Slaski Uniwersytet Medyczny w Katowicach, Katowice, Poland

The final, formatted version of the article will be published soon.

Background: Despite the use of different treatment regimens, patients with primary multimetastatic Ewing sarcoma disease have a dismal outcome. Lately, pazopanib has been proposed as an effective salvage regimen for soft tissue sarcoma (STS), including extraosseous Ewing sarcoma (ESS). Thus, we sought to evaluate this approach for young patients with primary multi-metastatic bone Ewing sarcoma.Materials and Methods: Eleven patients with primary multi-metastatic bone Ewing sarcoma (metastasis to the bone and/or bone marrow), received standard first-line treatment in parallel with pazopanib. All patients had standard tumor imaging and laboratory evaluation. All toxicities were documented.Results: Pazopanib was administered throughout the whole treatment period (paused during the surgical procedure) and after its completion, on average 1.7 years (range 0.9 to 5.1). At the time of the beginning of pazopanib, the median age was 14.2 years (range 5.1 to 17.8 years). The primary tumor was operated on in five patients. Ten patients received concurrent radiation therapy, and 3 autologous hematopoietic stem cell transplantation. Significant toxicities have not been observed. One patient (9.1%) progressed. Two patients had relapse (18.2%), and one patient died (9.1%). Ten patients (90.9%) are alive with a median time follow-up 2.6 years (range 1.2 to 9.2 years). The estimated 2-year event-free survival and overall survival for the whole group were 68.2% and 85.7%, respectively.Conclusions: Pazopanib was well-tolerated in young patients, even when it was administered with chemotherapy and radiation therapy together. Pazopanib turned out to be effective in patients with primary multi-metastatic Ewing sarcoma and particularly could be considered as an option for them. This regimen deserves further investigation.

Keywords: Pazopanib, metastatic Ewing sarcoma, Children, Treatment, Multi kinase inhibitors

Received: 24 Jun 2025; Accepted: 18 Aug 2025.

Copyright: © 2025 Raciborska, Bilska, Węcławek-Tompol, Sega-Pondel, Zelwiańska, Przybyszewski, Chaber, Tomaszewska, Antoniuk-Majchrzak and Koziński. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Anna Raciborska, Institute of Mother and Child, Warsaw, Poland

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