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软骨肉瘤——从分子病理学到新型疗法

Chondrosarcoma-from Molecular Pathology to Novel Therapies.

作者信息

Zając Agnieszka E, Kopeć Sylwia, Szostakowski Bartłomiej, Spałek Mateusz J, Fiedorowicz Michał, Bylina Elżbieta, Filipowicz Paulina, Szumera-Ciećkiewicz Anna, Tysarowski Andrzej, Czarnecka Anna M, Rutkowski Piotr

机构信息

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

Small Animal Magnetic Resonance Imaging Laboratory, Mossakowski Medical Research Institute, Polish Academy of Sciences, 02-106 Warsaw, Poland.

出版信息

Cancers (Basel). 2021 May 14;13(10):2390. doi: 10.3390/cancers13102390.

DOI:10.3390/cancers13102390
PMID:34069269
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8155983/
Abstract

Chondrosarcoma (CHS) is the second most common primary malignant bone sarcoma. Overall survival and prognosis of this tumor are various and often extreme, depending on histological grade and tumor subtype. CHS treatment is difficult, and surgery remains still the gold standard due to the resistance of this tumor to other therapeutic options. Considering the role of differentiation of CHS subtypes and the need to develop new treatment strategies, in this review, we introduced a multidisciplinary characterization of CHS from its pathology to therapies. We described the morphology of each subtype with the role of immunohistochemical markers in diagnostics of CHS. We also summarized the most frequently mutated genes and genome regions with altered pathways involved in the pathology of this tumor. Subsequently, we discussed imaging methods and the role of currently used therapies, including surgery and the limitations of chemo and radiotherapy. Finally, in this review, we presented novel targeted therapies, including those at ongoing clinical trials, which can be a potential future target in designing new therapeutics for patients with CHS.

摘要

软骨肉瘤（CHS）是第二常见的原发性恶性骨肿瘤。该肿瘤的总体生存率和预后各不相同，且往往差异极大，这取决于组织学分级和肿瘤亚型。CHS的治疗颇具难度，由于该肿瘤对其他治疗方案具有抗性，手术仍是金标准。鉴于CHS亚型分化的作用以及开发新治疗策略的必要性，在本综述中，我们介绍了从病理学到治疗方法的CHS多学科特征。我们描述了每种亚型的形态以及免疫组化标志物在CHS诊断中的作用。我们还总结了该肿瘤病理过程中最常发生突变的基因和基因组区域以及涉及的改变途径。随后，我们讨论了成像方法以及当前所用治疗方法（包括手术）的作用，以及化疗和放疗的局限性。最后，在本综述中，我们介绍了新型靶向治疗方法，包括正在进行临床试验的那些方法，这些方法可能成为未来为CHS患者设计新治疗方案的潜在靶点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/bc368d7f0c47/cancers-13-02390-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/51a6b5750d87/cancers-13-02390-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/282b376d0ad5/cancers-13-02390-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/db5d53668d5f/cancers-13-02390-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/51936289e271/cancers-13-02390-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/908d1aa2b310/cancers-13-02390-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/01d9bb2c7096/cancers-13-02390-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/fafc84e6eabc/cancers-13-02390-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/9ef1a99aa5fc/cancers-13-02390-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/bc368d7f0c47/cancers-13-02390-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/51a6b5750d87/cancers-13-02390-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/282b376d0ad5/cancers-13-02390-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/db5d53668d5f/cancers-13-02390-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/51936289e271/cancers-13-02390-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/908d1aa2b310/cancers-13-02390-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/01d9bb2c7096/cancers-13-02390-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/fafc84e6eabc/cancers-13-02390-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/9ef1a99aa5fc/cancers-13-02390-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14da/8155983/bc368d7f0c47/cancers-13-02390-g009.jpg

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NKX3.1 a useful marker for mesenchymal chondrosarcoma: An immunohistochemical study.
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