Bone Tumor Environment as a Potential Therapeutic Target in Ewing Sarcoma

Frontiers in Oncology
Françoise Redini, Dominique Heymann

Abstract

Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, Ewing sarcoma is an aggressive, rapidly fatal malignancy that mainly develops not only in osseous sites (85%) but also in extra-skeletal soft tissue. It spreads naturally to the lungs, bones, and bone marrow with poor prognosis in the two latter cases. Bone lesions from primary or secondary (metastases) tumors are characterized by extensive bone remodeling, more often due to osteolysis. Osteoclast activation and subsequent bone resorption are responsible for the clinical features of bone tumors, including pain, vertebral collapse, and spinal cord compression. Based on the "vicious cycle" concept of tumor cells and bone resorbing cells, drugs, which target osteoclasts, may be promising agents as adjuvant setting for treating bone tumors, including Ewing sarcoma. There is also increasing evidence that cellular and molecular protagonists present in the bone microenvironment play a part in establishing a favorable "niche" for tumor initiation and progression. The purpose of this review is to discuss the potential therapeutic value of drugs targeting the bone tumor microenvironment in Ewing sarcoma. The first part...Continue Reading

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Citations

Jan 11, 2017·Tissue Engineering. Part C, Methods·Aranzazu VillasanteGordana Vunjak-Novakovic
May 2, 2017·Frontiers in Endocrinology·Helen J Knowles
Sep 15, 2020·Journal of Extracellular Vesicles·Marta CollettiAngela Di Giannatale
Oct 4, 2019·Clinics and Practice·Girish Gulab MeshramKanwaljeet Singh Hura
Jul 3, 2021·Journal of Clinical Medicine·Sofía T MenéndezRené Rodríguez
Jul 31, 2021·Frontiers in Bioengineering and Biotechnology·Aranzazu VillasanteGordana Vunjak-Novakovic
Aug 3, 2018·ACS Nano·Carlos Rodríguez-NogalesMaría J Blanco-Prieto

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Methods Mentioned

BETA
amputation
GTPases
biopsies
xenografts

Clinical Trials Mentioned

NCT00020566
NCT00987636
NCT00691236
NCT00470223

Software Mentioned

EuroEWING

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