Abstract：
Primary pulmonary myxoid sarcoma is a rare lung sarcoma, mostly involving the central lung and harboring the EWSR1::CREB1 fusion. We report an exceptional case of primary pulmonary myxoid sarcoma arising in the peripheral lung and harboring an EWSR1::ATF1 gene fusion. A 67-year-old man presented with a solid nodule in the right lower lobe, and wedge resection was performed. Microscopically, the tumor consisted of reticular proliferation of uniform mildly atypical spindle cells within abundant myxoid stroma. Immunohistochemically, smooth muscle actin was positive but desmin was negative. Fluorescence in situ hybridization confirmed EWSR1 and ATF1 gene rearrangements. No recurrence was seen for 12 months. Pathological findings and gene rearrangements are important for the diagnosis of primary pulmonary myxoid sarcoma. Complete resection and careful observation are required.
摘要：
原发性肺粘液样肉瘤是一种罕见的肺肉瘤，主要累及中央肺，并有EWSR1::CREB1融合。我们报告了一例原发性肺黏液样肉瘤，起源于周围肺，并带有EWSR1::ATF1基因融合。一名67岁的男子，右下叶有一个实性结节，并进行楔形切除术。微观上,肿瘤由丰富的粘液样基质中均匀的轻度非典型梭形细胞的网状增殖组成。免疫组织化学，平滑肌肌动蛋白阳性，结蛋白阴性。荧光原位杂交证实了EWSR1和ATF1基因重排。12个月无复发。病理发现和基因重排对原发性肺粘液样肉瘤的诊断很重要。需要完全切除和仔细观察。
