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. Cancer 2007. © 2006 American Cancer Society.

No previous reports to date have characterized the national profiles of soft-tissue sarcomas (STSs). In the present study, we reviewed current practice for STSs in Japan using data from a nationwide organ-specific cancer registry for bone and soft-tissue tumors in Japan, the Bone and Soft Tissue Tumor (BSTT) Registry. In the registry, we identified 8228 patients with STSs during the period 2006-2012, and extracted data on patient demographics, treatment, and outcome at the last follow-up for each patient. Disease-specific survival was analyzed using the Cox proportional hazards model. STSs showed a slight male predilection. The age distribution had a single peak in the seventh decade, the proportion of elderly patients aged >60 years being approximately 53%. For most of the histologic subtypes, the most frequent tumor location was the lower extremity, whereas it was the trunk in patients with malignant peripheral nerve sheath tumor, dedifferentiated liposarcoma, and primitive neuroectodermal tumor. Based on data for 2432 patients with STSs, we found significant associations between disease-specific survival and age, sex, histologic subtype, tumor size, tumor depth, tumor location, additional surgery, limb salvage status, and surgical margin; elderly patients showed the poorest disease-specific survival. Using the BSTT Registry, this study has clarified the epidemiology, treatment, and prognosis of patients with STSs in Japan. Our experiences with the BSTT Registry will be of help to other countries where aging of the population is occurring. Continuous accumulation of clinical data in the BSTT Registry should provide more informative data on STSs, thus improving both the level of medical care offered by clinicians and the outcomes for patients through sharing of such data and promotion of clinical research.