医学
软组织肉瘤
舒尼替尼
放射治疗
肉瘤
透明细胞肉瘤
淋巴结切除术
免疫疗法
化疗
肿瘤科
癌症
病理
内科学
作者
Kazunari Ikuta,Yoshihiro Nishida,Shiro Imagama,Kazuhiro Tanaka,Toshifumi Ozaki
出处
期刊:Japanese Journal of Clinical Oncology
[Oxford University Press]
日期:2023-07-14
卷期号:53 (10): 899-904
被引量:1
摘要
Abstract 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.
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