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.

Seventy‐five consecutive patients with histologically confirmed CCS who received treatment between 1980 and 2004 were analyzed retrospectively.

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.

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. Cancer 2007. © 2006 American Cancer Society.