Abstract
INTRODUCTION: Clear cell sarcoma (CCS) of soft tissue is a rare type of soft tissue sarcoma affecting usually lower extremities in young adults. The main challenges in the management of this disease include difficulties in diagnosis, aggressiveness of the cancer with rapid progression, and inadequate treatment, especially in small centers with few cases. CASE PRESENTATION: We present a case of a young woman diagnosed with CCS of soft tissue, stage IV. The patient benefited from a multidisciplinary approach including radiation therapy, surgery, chemotherapy, and targeted therapy with disease progression regardless of the therapeutic act. Despite all the efforts, the patient died from complications overlapping progression of the disease. CONCLUSION: The rarity of this sarcoma limits the amount of information available on the diagnosis and treatment process. The particularity of this case is the difficulty met in maintaining the disease under control using all the resources available due to lack of compliance of the patient in carrying out amputation at diagnosis, thus modifying the entire treatment algorithm. Targeted therapy showed promising results in the literature, however in our case resulted in an unexpected, rare adverse event aggravating the patient's condition. In conclusion, patients with CCS should be referred to specialized centers for adequate multidisciplinary management and, if available, inclusion in clinical trials. New agents are needed to improve the survival of these patients.