Introduction: Clear cell sarcoma (CCS) is an ultra-rare soft tissue sarcoma (STS) with a poor prognosis due to propensity to metastasize and its low chemosensitivity. The standard treatment of localized CCS consists of wide surgical excision with or without additive radiotherapy. On the other hand, 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 therapies, in 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 ultra-rare sarcoma and identify potential molecular targets.
Clear cell sarcoma: state-of-the art and perspectives
Dei Tos, Angelo;
2023
Abstract
Introduction: Clear cell sarcoma (CCS) is an ultra-rare soft tissue sarcoma (STS) with a poor prognosis due to propensity to metastasize and its low chemosensitivity. The standard treatment of localized CCS consists of wide surgical excision with or without additive radiotherapy. On the other hand, 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 therapies, in 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 ultra-rare sarcoma and identify potential molecular targets.Pubblicazioni consigliate
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