Clear cell sarcoma: 20 years of experience at Instituto Nacional de Enfermedades Neoplasicas (INEN)

Abstract

Background: Clear cell sarcoma (CCS) of soft tissue is a rare disease with a high risk of metastasis to regional lymph nodes and distant organs and a poor survival rate. The aim of this study is to determine the rate of lymph node involvement, the effectiveness of treatment, the risk of recurrence and progression after surgery.

Methods: We collected data from twenty patients diagnosed with CCS and treated in our institute, between 1998 and 2018. Subsequently, survival rates were determined according to local, regional and distant involvement, as well as the prognostic factors.

Results: Twenty patients with CCS were included. The 2-year survival rate was 20%, and the 5-year survival rate was 5%. Patients with CCS with local stage and with tumor size < 5.0 cm were more likely to have a good survival rate.

Conclusion: The initial management is crucial for the prognosis of the disease, with surgery being the mainstay of treatment. This study revealed a high rate of lymph node metastasis, so regional lymph node dissection should be done. Finally, the role of chemotherapy and/or radiotherapy for survival is still unclear.

Keywords: Sarcoma; clear cell sarcoma; soft tissue tumors