Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients

BACKGROUND: Clear cell sarcoma (CCS) of tendons and aponeuroses (malignant melanoma of soft parts) is a rare melanocytic soft tissue sarcoma. The objective of this study was to determine the clinical features, prognostic factors, and optimal treatment policy for patients with this rare disease. METHODS: Seventy-five consecutive patients with histologically confirmed CCS who received treatment between 1980 and 2004 were analyzed retrospectively. RESULTS: There were 41 men and 34 women, and the median age was 36 years. Sixty-five tumors were located in the extremities, and 10 tumors were located in the trunk. The median tumor size was 4 cm. Seventy-one patients underwent surgical excision, and 56 patients received chemotherapy. Sixteen patients developed local recurrences, and 52 patients developed metastasis. The overall patient survival rates was 47% at 5 years and 36% at 10 years. Univariate analysis showed that sex (P = .018), tumor size (P = .001), tumor depth (P = .002), TNM classification (P = .001), and surgical margin (P = .042) were significant prognostic factors. Among the 52 patients who presented with localized disease, sex (P = .023), tumor size (P = .002), tumor depth (P = .011), TNM classification (P = .004), and chemotherapy (P = .032) were identified as significant prognostic factors. Multivariate analysis showed that tumor size remained an independent prognostic factor in both groups. CONCLUSIONS: The current results supported the contention that early diagnosis and initial wide excision are essential for a favorable outcome of CCS. The role of chemotherapy for CCS should be investigated further.