Surgically Treated Clear Cell Sarcomas - What Influences Outcomes?

Abstract

Clear cell sarcoma (CCS) also called as melanoma of soft parts is a rare malignant soft-tissue tumor with melanocytic differentiation, primarily located in deep soft tissue and has preference for lymph node and pulmonary metastasis. Metastatic patients have poor oncologic prognosis and so far, no adjuvant treatment seems to be effective in these cases. All cases were retrieved from our prospectively maintained surgical database. Twenty-nine patients (14 males,15 females) with a mean age of 34 years (13-69 years) were operated between 2004 and 2020. Overall survival and recurrence free survival were evaluated. At mean follow-up of 45 months, out of 29 cases, 2 lost to follow-up, 6 patients had nodal metastasis, 3 had lung metastasis, one had both at presentation, 15 patients had died due to disease and 12 are alive. Nodal metastasis rate was 24% (7/29). Positive margin is observed in 6 (19%). 24 patients had recurrence of which 6 had both local and distant. The 5-year OS, LRFS, DRFS were 53%, 56%, 35% respectively at 5 years. Survival rates were significantly poor in patients with metastasis at presentation, 66% vs. 25% (p = 0.016). CCS is an aggressive soft tissue malignancy with high propensity for metastasis. The overall survival of CCS is poor. Metastasis at presentation negatively impacts on survival. Distant recurrences, especially nodal recurrences are not uncommon and complete surgical resection of all recurrences if possible is advisable. Research to develop new chemotherapeutic agents and targeted therapies may help improve the prognosis of CCS.