Metastatic clear cell sarcoma of the pancreas: An overview

Clear cell sarcoma (CCS) is a rare soft-tissue sarcoma that accounts for less than 1% of all cases and was originally reported in 1965. The incidence of CCS is estimated to be approximately 0.014/100000 depending on the surveillance, epidemiology and end results databases. CCS is a highly invasive type that mainly metastasizes to the lungs, followed by the bones and brain; however, pancreatic metastasis is relatively rare. It has a high probability of recurrence or metastasis and has a poor prognosis with a high mortality rate. Finally, even after recovery, it is fundamental to keep regular postoperative follow-up for CCS patients.