Department of Respiratory Medicine, Sasebo City General Hospital, Nagasaki, Japan.
Department of Respiratory Medicine, Senju Hospital, Nagasaki, Japan.
Thorac Cancer. 2022 Oct;13(19):2812-2816. doi: 10.1111/1759-7714.14613. Epub 2022 Aug 16.
Extraskeletal myxoid chondrosarcoma (EMCS) is an undifferentiated mesenchymal malignancy; however, its immune microenvironment remains to be elucidated. The case of a 34-year-old woman who developed EMCS metastasizing to the pleura is presented here. The pleural EMCS showed hypervascularity, absent PD-L1 expression, and a lack of tumor mutational burden and pathogenic variants. Immunohistological examination of the pleural lesions showed predominant M2 macrophages and sparse CD8 T cells. EMCS and the tumor stroma were positive for transforming growth factor-β1 (TGF-β1) and vascular endothelial growth factor (VEGF). In contrast, a small number of the stromal vessels were positive for hypoxia inducible factor-1α (HIF-1α). TGF-β1 and VEGF in the tumor stroma and low antigenicity of the tumor cells may help explain how EMCS induced the immunosuppressive microenvironment. These findings may encourage investigators to explore novel combined immunotherapy for EMCS, such as TGF-β1 and VEGF inhibitors, and specific therapy for enhancing tumor antigens.
骨外黏液样软骨肉瘤（EMCS）是一种未分化的间充质恶性肿瘤；然而，其免疫微环境仍有待阐明。本文报告了一例 34 岁女性发生胸膜转移的 EMCS 病例。胸膜 EMCS 表现为血管丰富，PD-L1 表达缺失，且肿瘤突变负担和致病性变异缺失。胸膜病变的免疫组织化学检查显示以 M2 巨噬细胞为主，CD8 T 细胞稀疏。EMCS 和肿瘤基质均呈转化生长因子-β1（TGF-β1）和血管内皮生长因子（VEGF）阳性。相比之下，少数基质血管呈缺氧诱导因子-1α（HIF-1α）阳性。肿瘤基质中的 TGF-β1 和 VEGF 以及肿瘤细胞的低抗原性可能有助于解释 EMCS 如何诱导免疫抑制微环境。这些发现可能鼓励研究人员探索 EMCS 的新型联合免疫疗法，如 TGF-β1 和 VEGF 抑制剂，以及增强肿瘤抗原的特异性治疗。
