Clear cell sarcoma: state-of-the art and perspectives.

IF 2.9 3区医学 Q2 ONCOLOGY

Expert Review of Anticancer Therapy Pub Date : 2023-03-01 DOI:10.1080/14737140.2023.2183846

Laureline Wetterwald, Nicolò Riggi, Anastasios Kyriazoglou, Giovanni Dei Tos, Angelo Dei Tos, Antonia Digklia

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引用次数: 4

Abstract

Introduction: Clear cell sarcoma (CCS) is an ultrarare soft tissue sarcoma (STS) with a poor prognosis due to its propensity to metastasize and its low chemosensitivity. The standard treatment of localized CCS consists of wide surgical excision with or without additive radiotherapy. However, unresectable CCS is generally treated with conventional systemic therapies available for treatment of STS despite the weak scientific evidence to support its use.

Areas covered: In this review, we discuss the clinicopathologic characteristics of CSS, as well as the current treatment landscape and future therapeutic approaches.

Expert opinion: The current treatment strategy of advanced CCSs, based on STSs regimens, shows a lack of effective options. Combination therapiesin particular, the association of immunotherapy and TKIs, represent a promising approach. Translational studies are needed in order to decipher the regulatory mechanisms involved in the oncogenesis of this ultrarare sarcoma and identify potential molecular targets.

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透明细胞肉瘤:现状和观点。

简介:透明细胞肉瘤(CCS)是一种超罕见的软组织肉瘤(STS)，由于其易转移和化疗敏感性低，预后较差。局限性CCS的标准治疗包括广泛的手术切除和或不加放疗。然而，不可切除的CCS通常采用常规的全身疗法治疗STS，尽管支持其使用的科学证据不足。涵盖领域:在这篇综述中，我们讨论了CSS的临床病理特征，以及目前的治疗前景和未来的治疗方法。专家意见:目前基于STSs方案的晚期CCSs治疗策略显示缺乏有效的选择。联合治疗，特别是免疫治疗和TKIs的结合，是一种很有前途的方法。翻译研究是必要的，以破译参与肿瘤发生的这种罕见肉瘤的调控机制，并确定潜在的分子靶点。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Expert Review of Anticancer Therapy 医学-肿瘤学

CiteScore

5.10

自引率

3.00%

发文量

100

审稿时长

4-8 weeks

期刊介绍： Expert Review of Anticancer Therapy (ISSN 1473-7140) provides expert appraisal and commentary on the major trends in cancer care and highlights the performance of new therapeutic and diagnostic approaches. Coverage includes tumor management, novel medicines, anticancer agents and chemotherapy, biological therapy, cancer vaccines, therapeutic indications, biomarkers and diagnostics, and treatment guidelines. All articles are subject to rigorous peer-review, and the journal makes an essential contribution to decision-making in cancer care. Comprehensive coverage in each review is complemented by the unique Expert Review format and includes the following sections: Expert Opinion - a personal view of the data presented in the article, a discussion on the developments that are likely to be important in the future, and the avenues of research likely to become exciting as further studies yield more detailed results Article Highlights – an executive summary of the author’s most critical points.