Background: 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.
Conclusion: 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.
Keywords: EWSR1 gene; FISH; Primary pulmonary myxoid sarcoma; immunohistochemistry.
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