ABSTRACT
Sarcomas are an unusual group of tumors, accounting for ~1% of cancer in adults. Immunotherapy has been shown to be a potential therapeutic option for the management of patients with cancer. However, there is still insufficient information on the action of immunotherapy on sarcomas. A 16-year-old male patient, diagnosed in December 2013 with grade III soft-tissue sarcoma in the right arm, was admitted to a private oncology service after relapse following surgical treatment. The patient underwent chemotherapy with ifosfamide plus adriamycin for 4 cycles, associated with adjuvant radiotherapy, followed by a new resection to remove the residual lesion. A year later, imaging tests identified pulmonary micronodules, and a new resection was performed. After immunohistochemical evaluation of biopsy, a large presence of programmed cell death 1 ligand 1 (PD-L1) marker was identified in tumor tissue and immunotherapy with nivolumab was performed. At present, the patient is in immunotherapeutic treatment (42 cycles), presenting an excellent general condition and without any symptoms, and a decrease in neoplastic lung masses. The literature recommends three cycles of anthracycline plus ifosfamide as adjuvant therapy to surgical treatment. Combined surgery plus adjuvant therapy has shown benefits in malignant tumors. Immunotherapy is an important therapeutic option for soft-tissue sarcomas with high programmed cell death protein 1 (PD-1)/PD-L1 expression. Treatment for high grade soft tissue sarcoma (STS) is still limited, due to tumor heterogeneity, and further studies are needed to consolidate the possibility of using immunotherapy to treat these neoplasms. When significant levels of specific biomarkers are present in tumor tissue, immunotherapy may be beneficial as shown by the present case report.