Clear-cell sarcoma of tendons and aponeuroses:: three case reports

Clear-cell sarcoma (CCS) of tendons and aponeuroses is a rare malignant tumor representing about 1% of soft tissue tumors. Preferentially observed in young adults in the second or third decade, the tumor generally develops in the limbs. Only 2% of SCC of tendons and aponeuroses have been reported in children less than 10 years of age. This slowly progressive tumor usually forms a painless mass. The tumor increases in size followed by metastatic dissemination to lymph nodes and the lungs. The prognosis is related to tumor size. At the present time, the recognized limit is greater than 5 cm. Early diagnosis must be achieved to enable effective treatment by carcinological surgical resection. We report the three cases of CCS of tendons and aponeuroses observed at the Strasbourg University hospital over a 35-year period. Each case had a special clinical presentation. The first patient, treated in 1967, presented tumor bone lysis on the plain x-ray, an observation rarely reported in the literature. In the second patient, treated in 2002, the tumor was discovered after trauma. This patient developed skin ulceration associated with paraplegia secondary to metastatic thoracic cord compression. The third case occurred in a 12-year-old girl, treated in 2002.