Clear cell sarcoma is a rare malignant soft tissue neoplasm with a predilection for arising deep soft tissues adjacent to tendons and aponeuroses and fascial structures. This report presents a 32-year-old woman with low back pain and swelling, night sweat and lateral numbness in her right thigh for three months. MRI revealed a 4.0 x 5.8 cm vertebral lesion on the right lumbar region, without characteristics of a melanin rich tumor. Immunohistochemistry and fluorescence in situ hybridization confirmed diagnosis of clear cell sarcoma. The patient underwent a chemotherapy protocol. At four months post-surgery, CT revealed a soft tissue lesion in the right L3, suspected to be either a cancer relapse or harmless post-surgery lesion. Radiotherapy was performed in precaution of a relapse. At eleven months post-surgery, tumor relapse and multiple bone metastasis were observed. Combination of imaging methods improves diagnostics and subsequently differentiate clear cell sarcoma from other pathologies.
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