Abstract
Epithelioid sarcoma (ES) is a rare soft tissue malignant tumor with an uncertain histogenetic origin. It usually arises in soft tissues of the extremities, while ES in adrenal gland is extremely rare. There is no special clinical manifestation in the early stage, so it may be misdiagnosed and delay the treatment. We reported a 69-year-old male with an adrenal ES. The tumor was completely resected, and two months later, positron emission tomography-computed tomography(PET/CT) noted recurrence at the tumor bed and multiple metastases. The patient has been treated with chemotherapy with good effects. We summarize the radiological findings and immunohistochemical indexes of primary epithelioid sarcoma of adrenal gland, which may be useful to promote disease awareness and help to distinguish among other lesions.