Objective: To explore the clinical features, prognosis, and optimal treatment strategy of clear cell sarcoma (CCS).
Methods: Nineteen patients, 12 males and 7 females, aged 30. 9, with histologically confirmed CCS, 3 with tumors > or =5 cm and 16 with tumors <5 cm, 5 tumors being located in the upper extremity, 2 in the hand, 6 in the lower extremity, 2 in the foot, 3 in the trunk, and 1 in the head, were hospitalized from March 1973 to March 2007. The primary tumors of all the cases were marginally excised before admission and 10 cases had local relapse at admission. Two presented local lymph node metastasis and 2 presented distant metastasis. Ten patients with tumor relapse underwent re-operation. Eight patients received chemotherapy, 8 radiotherapy and 1 combined chemotherapy and radiotherapy. The patients were followed up for 51.4 months (3-144 months).
Results: Tumor recurrence occurred in 1 of the 10 patients who received re-operation. 3 patients developed lymph node metastasis and 2 developed pulmonary metastasis. Of the 9 patients who did not undergo re-operation 7 developed lymph node metastasis, 6 developed pulmonary metastasis and 6 died. The overall 5-year survival rate was 75.2% and the 10-year survival rate was 37.5%.
Conclusion: CCS is a rare and high grade soft tissue sarcoma with high incidence rates of local recurrence and metastasis, and poor prognosis. The role of chemotherapy and radiotherapy for CCS should be investigated further. The best choice after local recurrence is re-operation.