Abstract
BACKGROUND: Clear cell sarcoma of tendons and aponeuroses (CCSTA) appears usually in the extremities and rarely in the trunk. CASE PRESENTATION: We present an unusual case of CCSTA overlying the scapular region and with secondary osseous extension in the lower scapula. The patient underwent a wide local excision with removal of the tumor and the lower two thirds of the scapula. He had no local recurrences but he developed lung metastases after 5 months in spite of postoperative chemotherapy. He finally died ten months later. CONCLUSION: The patients with CCSTA have a variable unpredictable course. Despite treatment the overall prognosis is poor.