Clear Cell Sarcoma of Tendons and Aponeuroses

Abstract

Fifty-eight previously unreported cases of clear cell sarcoma of tendons and apo neuroses occurring in 29 males and 29 females with a median age of 31 years are presented. The distal extremities were most frequently involved (58%), followed by the limbs and limb girdles (33%), and trunk (9%). Median tumor size was 2.5 cm (range, 0.6-9 cm). Kaplan-Meier estimates revealed a 5-year survival rate of 63%. Eleven of 43 patients (26%) had local recurrences at a median interval of 33 months. Metastases occurred in 20 of 45 cases (44%) at a median interval of 26 months; 19 of 20 patients with metastases died of the disease. The most common metastatic site was the lungs (11 cases), followed by bone (9 cases), and lymph nodes (8 cases). Clear cell sarcoma had a stereotypic morphology consisting of packeted arrangements of uniform, round to fusiform cells with eosinophilic to clear glycogen-rich cytoplasm, and prominent nucleoli, as well as multinucleated tumor cells with a wreath-like arrangement of nuclei. S100 protein was detected in all 44 cases studied, vimentin in 36, and HMB45 stained 36. Twenty-six cases were studied with antibodies directed against proliferating cell nuclear antigen. Neither the intensity of proliferating cell nuclear antigen staining, percentage of positive cells, nor the combined score of these two variables corresponded with mitotic rate or survival. Increasing size (as a continu ous variable) and necrosis were found to be independent adverse prognostic factors by multivariate analysis. Int J Surg Pathol 1 (2):89-100, 1993