Clinicopathological analysis of myxoid proximal-type epithelioid sarcoma

Proximal-type epithelioid sarcoma (ES) with a diffuse myxoid stroma is rare. Here, we report the case of a 33-year-old man with a perineal mass. Imaging showed the presence of a poorly demarcated 6.9 × 5.3-cm mass in the subcutaneous perineal region. Macroscopic examination showed that the resected tissues were partially necrotic. Histological examination showed that the tumor comprised numerous large or pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. A clear background of necrosis and inflammatory exudates was also present. Immunohistochemical examination showed that the tumor cells were positive for vimentin and CD34 - both of which were expressed throughout the cytoplasm - but typically did not express nuclear INI1 (SMARCB1). Hematoxylin-eosin staining (HE staining) showed that the mucin content of the tumor was approximately 80%. The patient was diagnosed with proximal-type ES with myxoid features. The patient died due to disease progression after 2 months of follow-up and without undergoing further treatment in our department. To our knowledge, only 2 cases of proximal-type ES with diffuse myxoid stroma have been reported. Proximal-type ES is rare, and this is the first case report of proximal ES with myxoid features in the perineal area.