Mesenchymal Chondrosarcoma from Diagnosis to Clinical Trials

Author:

Dudzisz-Śledź Monika1ORCID,Kondracka Monika12,Rudzińska Monika12,Zając Agnieszka E.1ORCID,Firlej Wiktoria12,Sulejczak Dorota3ORCID,Borkowska Aneta1,Szostakowski Bartłomiej1ORCID,Szumera-Ciećkiewicz Anna45ORCID,Piątkowski Jakub6,Rutkowski Piotr1ORCID,Czarnecka Anna M.13ORCID

Affiliation:

1. Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland

2. Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland

3. Department of Experimental Pharmacology, Mossakowski Medical Research Centre Polish Academy of Sciences, 02-106 Warsaw, Poland

4. Department of Pathology, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland

5. Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, 02-776 Warsaw, Poland

6. Institute of Genetics and Biotechnology, Faculty of Biology, University of Warsaw, 02-106 Warsaw, Poland

Abstract

Mesenchymal chondrosarcoma (MCS) is a rare subtype of chondrosarcoma with a poor prognosis. Although these tumors are sensitive to radiotherapy/chemotherapy, the standard treatment for localized MCS is only surgical resection, and there are no established treatment guidelines for patients with advanced and metastatic MCS. Due to the low incidence of MCS, the pathology of these tumors is still unknown, and other therapeutic options are lacking. Some studies show the potential role of the PDGF/PPI3K/AKT, PKC/RAF/MEK/ERK, and pRB pathways, and BCL2 overexpression in the pathogenesis of MCS. These findings provide an opportunity to use protein kinases and BCL2 inhibitors as potential therapy in MCS. In this review, we summarize the current knowledge about MCS diagnosis and treatment options. We show the immunological and molecular biomarkers used in the diagnosis of MCS. In addition, we discuss the known prognostic and predictive factors in MCS. Finally, we present the novel trends, including targeted therapies and ongoing clinical trials using protein kinase inhibitors and the death receptor 5 (DR5) agonist, which may be the focus of future MCS treatment studies.

Funder

National Science Centre

Publisher

MDPI AG

Subject

Cancer Research,Oncology

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