Clear cell sarcoma is an uncommon malignant soft tissue tumor. It can cause some difficulties in differential diagnosis histopathologically. So, distinction of clear cell sarcoma from synovial sarcoma, fibrosarcoma, malignant nerve sheath tumor, desmoplastic malignant melanoma and renal cell carcinoma is important.

The patient is twenty years old male. He has described swelling and pain on the left foot heel for about two months. Biopsy was performed the patient in the orthopaedic clinic of the another hospital. Biopsy was reported as synovial sarcoma by histopathological examination. The patient was referred to department of orthopaedics of the Gulhane Military Medical Academy after this diagnosis. Slides, blocks and tissue specimens of the patient was consultated the department of the pathology of the Gulhane Military Medical Academy. The mass which was 6x4x3,5 cm in dimension was determined in magnetic resonance imaging. Tumor had formed atypic cells which had consisted of nests and fassicules histopathologically. There was expression of S 100 protein, HMB 45 and vimentin in the neoplastic cells. But, no expression of cytokeratin and desmin. The left foot amputation was performed the patient who was diagnosed as clear cell sarcoma with this findings. Disseminated metastases was detected after six weeks from the operation. A palliative radiotherapy was performed the thoracal region and chemotherapy was planned. The patient died before the chemotherapy.

A clear cell sarcoma case which has shown disseminated metastases in a short time, has presented.

Keywords: Clear Cell Sarcoma, Soft Tissue