Objective: Our study was conducted to summarize the clinical and radiologic presentations and imaging features of proximal-type epithelioid sarcoma, a rare aggressive soft-tissue sarcoma, in 16 patients.
Conclusion: The chest wall, inguinal region, thigh, and perineum were the most common sites of the disease. Tumors exhibited multilobulated contours in 87.5% of the patients, which may be suggestive of this tumor type. Nodal involvement was identified on CT and MR imaging and confirmed pathologically in 62.5% of the patients. Although CT or MR findings are nonspecific, proximal-type epithelioid sarcoma shows a multinodular configuration and admixes with regional lymph node metastasis on imaging studies.