Wu Yingying, Luo Yanfang, Gong Youjie, Yang Ruixue, Ding Li, Guo Bingqin
Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College Bengbu, Anhui Province, China.
Department of Pathology, Bengbu Medical College Bengbu, Anhui Province, China.
Int J Clin Exp Pathol. 2021 Feb 1;14(2):230-237. eCollection 2021.
Primary pulmonary sarcoma is extremely rare and mostly metastatic, and primary pulmonary myxoid sarcoma PPMS is a rare low-grade malignant sarcoma. The clinical manifestations of PPMS patients are relatively non-specific, sometimes found by physical examination. We report a case designed to explore the clinicopathologic features, diagnosis, and differential diagnosis of primary pulmonary myxoid sarcoma (PPMS). A 44-year-old man was found to have a primary myxoid sarcoma in the upper right lung on physical examination. The patient did not have any symptoms of discomfort. Histologically, the tumors had well-defined borders, and with grayish-white or grayish red cut surfaces. Under the microscope, the tumor cells were composed of oval and spindle cells arranged in a network or strips in a mucus-like stroma. Immunohistochemically, neoplastic cells showed diffuse and strong vimentin expression and focal weak EMA, and Bcl-6 staining. The expression of AE1/AE3, ALK, CD34, CD68, SMA, and CD99 were all negative. The Ki-67 index was low.
PPMS is a rare low-grade malignant primary pulmonary sarcoma without characteristic clinical symptoms and difficult to diagnose. It is mainly diagnosed by immunohistochemistry and genetic testing.
原发性肺肉瘤极为罕见，多为转移性，而原发性肺黏液样肉瘤（PPMS）是一种罕见的低级别恶性肉瘤。PPMS患者的临床表现相对不具特异性，有时通过体格检查发现。我们报告一例旨在探讨原发性肺黏液样肉瘤（PPMS）的临床病理特征、诊断及鉴别诊断。一名44岁男性在体格检查时发现右上肺有原发性黏液样肉瘤。患者无任何不适症状。组织学上，肿瘤边界清晰，切面呈灰白色或灰红色。显微镜下，肿瘤细胞由椭圆形和梭形细胞组成，在黏液样基质中呈网状或条索状排列。免疫组化显示，肿瘤细胞弥漫性强表达波形蛋白，局灶性弱表达上皮膜抗原（EMA）和Bcl-6染色。AE1/AE3、间变性淋巴瘤激酶（ALK）、CD34、CD68、平滑肌肌动蛋白（SMA）和CD99的表达均为阴性。Ki-67指数较低。
PPMS是一种罕见的低级别恶性原发性肺肉瘤，无特征性临床症状，诊断困难。主要通过免疫组化和基因检测进行诊断。
