Kim Jong Hun, Kim Kyoung Min
Department of Thoracic and Cardiovascular Surgery, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Jeollabuk 54907, Republic of Korea.
Department of Pathology, Institute for Medical Sciences of Jeonbuk National University, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Jeollabuk 54907, Republic of Korea.
Oncol Lett. 2025 Apr 11;29(6):289. doi: 10.3892/ol.2025.15036. eCollection 2025 Jun.
Pulmonary myxoid sarcoma is an rare and poorly understood malignant neoplasm that primarily arises within the pulmonary tissue. Characterized by its distinctive histological features of myxoid stroma and spindle-shaped cells, this neoplasm poses significant diagnostic and therapeutic challenges due to its rarity and the non-specific nature of its clinical presentation. Current knowledge regarding the pathogenesis, optimal therapeutic strategies and prognostic factors for pulmonary myxoid sarcoma remains limited, primarily due to the scarcity of reported cases and comprehensive studies. The present study reports a case of pulmonary myxoid sarcoma. A 41-year-old male was admitted to the Jeonbuk National University Hospital due to a pulmonary mass in the left lower lobe discovered during a routine health check-up. A CT scan performed at our hospital revealed a nodule ~1 cm in size in the mediobasal segment of the left lower lobe, with relatively well-defined margins and significant enhancement. A wedge resection was performed for diagnosis and treatment, and frozen section examination showed a high likelihood of pleomorphic adenoma. The histological findings of the permanent section examination revealed an abundant myxoid matrix with embedded spindle, stellate and rounded/epithelioid cells arranged in a reticular pattern. The tumor cells exhibited mild to moderate cellular atypia, with rare mitotic figures. Immunohistochemistry showed positive staining for vimentin and negative findings for myoepithelial cell markers such as calponin, high-molecular weight cytokeratin and p63. The presence of the EWSR1-CREB1 fusion was confirmed through fluorescence in situ hybridization and reverse transcription-PCR analyses. Based on these findings, the nodule was diagnosed as pulmonary myxoid sarcoma.
肺黏液样肉瘤是一种罕见且了解甚少的恶性肿瘤，主要起源于肺组织。该肿瘤以其黏液样基质和梭形细胞的独特组织学特征为特点，由于其罕见性和临床表现的非特异性，在诊断和治疗方面面临重大挑战。目前关于肺黏液样肉瘤的发病机制、最佳治疗策略和预后因素的知识仍然有限，主要原因是报告的病例和综合研究较少。本研究报告了一例肺黏液样肉瘤病例。一名41岁男性因在常规健康检查中发现左肺下叶肺部肿块入住全北国立大学医院。我院进行的CT扫描显示左肺下叶内基底段有一个大小约1cm的结节，边界相对清晰，有明显强化。为进行诊断和治疗实施了楔形切除术，冰冻切片检查显示多形性腺瘤的可能性很大。永久切片检查的组织学结果显示有丰富的黏液样基质，其中嵌入呈网状排列的梭形、星状和圆形/上皮样细胞。肿瘤细胞表现出轻度至中度细胞异型性，有罕见的有丝分裂象。免疫组织化学显示波形蛋白染色阳性，而肌上皮细胞标志物如钙调蛋白、高分子量细胞角蛋白和p63染色阴性。通过荧光原位杂交和逆转录-聚合酶链反应分析证实了EWSR1-CREB1融合的存在。基于这些发现，该结节被诊断为肺黏液样肉瘤。
