Department of Orthopedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya 466-8550, Japan.
Department of Rehabilitation, Nagoya University Hospital, 65 Tsurumai, Showa, Nagoya 466-8550, Japan.
Jpn J Clin Oncol. 2023 Oct 4;53(10):899-904. doi: 10.1093/jjco/hyad083.
Clear cell sarcoma (CCS) is a rare melanocytic soft tissue sarcoma with a high propensity for lymphatic metastasis and poor prognosis. It is characterized by the translocation of t (12;22), resulting in the rearrangement of the EWSR1 gene and overexpression of MET. Despite improvements in the diagnosis and treatment of soft tissue sarcomas, the management of CCSs remains challenging owing to their rarity, unique biological behaviour and limited understanding of their molecular pathogenesis. The standard treatment for localized CCSs is surgical excision with negative margins. However, there is an ongoing debate regarding the role of adjuvant chemotherapy, radiotherapy and lymphadenectomy in the management of this disease. CCSs are usually resistant to conventional chemotherapy. Targeted therapies, such as sunitinib and MET inhibitors, may provide promising results. Immunotherapy, particularly immune checkpoint inhibitors, is currently under investigation as a potential treatment option for CCSs. Further research is needed to better understand the biology of CCSs and develop effective therapeutic strategies. The purpose of this review is to provide a comprehensive overview of current knowledge and advances in the diagnosis and treatment of CCSs.
透明细胞肉瘤（CCS）是一种罕见的黑色素细胞软组织肉瘤，具有较高的淋巴转移倾向和不良预后。其特征在于 t（12；22）易位，导致 EWSR1 基因重排和 MET 过表达。尽管软组织肉瘤的诊断和治疗有所改善，但由于 CCS 的罕见性、独特的生物学行为以及对其分子发病机制的了解有限，其管理仍然具有挑战性。局限性 CCS 的标准治疗方法是手术切除并保证切缘阴性。然而，在辅助化疗、放疗和淋巴结清扫术在该疾病管理中的作用方面仍存在争议。CCS 通常对常规化疗具有耐药性。靶向治疗，如舒尼替尼和 MET 抑制剂，可能会提供有希望的结果。免疫疗法，特别是免疫检查点抑制剂，目前正在作为 CCS 的潜在治疗选择进行研究。需要进一步的研究来更好地了解 CCS 的生物学特性并开发有效的治疗策略。本文的目的是全面概述 CCS 的诊断和治疗方面的现有知识和进展。
