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Osteosarcoma (OS) and Ewing Sarcoma (ES) are the two most frequent malignant bone tumors in children, adolescents and young adults. In case of disease recurrence, both are characterized by an aggressive behaviour and a relatively poor overall survival rate, with approximately a third of patients having a long-term disease-free survival. In case of recurrent or refractory (R/R) disease, the therapeutic strategy should be discussed in multidisciplinary staff meetings with expertise in bone sarcoma management. The standard management of R/R OS depends on the disease-free interval and the number and sites of metastases and is primarily surgical in patients with isolated lung metastases or local relapse. On the other hand, conventional chemotherapy remains the standard for R/R ES and include high-dose ifosfamide, cyclophosphamide with topotecan and irinotecan with temozolomide.
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http://dx.doi.org/10.1016/j.bulcan.2024.03.010 | DOI Listing |
Eur J Surg Oncol
August 2025
Royal Orthopaedic Hospital, Birmingham, United Kingdom.
Introduction: Pelvic bone sarcomas are rare, heterogeneous malignancies that present significant diagnostic and therapeutic challenges. Despite advances in imaging, surgical navigation, and multidisciplinary care, it remains unclear whether these innovations have improved outcomes across all histiotypes.
Material And Methods: We conducted a retrospective cohort study of 475 patients surgically treated for primary pelvic bone sarcomas between 2003 and 2022.
Cancer Res Commun
August 2025
Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
Unlabelled: Ewing sarcoma is an aggressive cancer diagnosed in adolescents and young adults. Inhibition of TGFβ is being tested in limited clinical trials for relapsed Ewing sarcoma. TGFβ is an immunosuppressive cytokine that exists in latent and active states.
View Article and Find Full Text PDFFolia Med (Plovdiv)
August 2025
Medical University of Sofia, Sofia, Bulgaria.
Ewing sarcoma is a rare and highly aggressive primary malignant bone tumor. Early diagnosis is difficult, especially in the early stages of the disease, which often results in delays in diagnosis.
View Article and Find Full Text PDFPediatr Blood Cancer
October 2025
Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Outcomes for relapsed Ewing sarcoma remain consistently poor. Continued efforts to consider creative new approaches for the treatment of relapsed Ewing sarcoma are needed. The Children's Oncology Group Bone Tumor Committee convened a New Strategies for Ewing Sarcoma Task Force to systematically evaluate agents for inclusion in future Phase II or III clinical trials for relapsed Ewing sarcoma.
View Article and Find Full Text PDFAnticancer Res
September 2025
Department of Regional Gynecologic Oncology, Fukushima Medical University, Fukushima, Japan.
Background/aim: Primary uterine Ewing's Sarcoma is an extremely rare and aggressive tumor. Due to its rarity, there is no established standard treatment, and most cases involve radical surgery, often compromising fertility and ovarian function. Recent advances in multimodal therapy have introduced fertility-preserving approaches for young patients.
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