Abstract
Clear cell sarcoma (CCS) is a rare and highly malignant soft-tissue sarcoma (STS) constituting 1% of all STS. It most often appears in the soft tissue closely assocíated with tendons, aponeuroses or fascial tissue of the distal extremities. It shares features of melanomas, hence is dubbed as a soft-tissue melanoma or clear cell sarcoma of the tendons and aponeuroses (CCSTA). CCS differs from the more common STS by its more aggressive growth and greater propensity to metastasise to lymph nodes, bones and lung. On a molecular basis, CCS is characterised by the chromosomal translocation.
Clinically, the tumor is firm, slowly growing and painless in half of the cases, thus rarely awakening suspicion of a STS. Thus, the diagnosis is difficult. Furthermore, on an MRI the tumor exhibits mostly a homogenous mass with a higher signal intensity as compared to muscle on T1-weighted images and implies a benign tumor.
The most important treatment is a wide resection to ensure local control. Little is known about neo- and/or adjuvant chemotherapy or radiation. Regarding chemotherapy different substances (doxorubicin, ifosfamide, cisplatin, mesna, dacarbazine, cyclophosphamide) have been administered under different conditions (single or combined substances, different doses, neo- or adjuvant). There are also some reports displaying the advantages of caffeine-assisted chemotherapy.
Under similar different circumstances radiation therapy was applied.
Most important prognostic factors are the presence of tumor necroses, size and regional lymph node metastases, local recurrence and distant metastases.
The reported 5-year survival ranges from 48% to 68%, the 10-year survival from 36% to 41%. Summarising all reports there is a need at least for a retrospective study to gather information about more patients.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
