2018 Volume 55 Issue 5 Pages 421-426
A 14-year-old boy was referred to our center for further treatment of a mediastinal mass with associated symptoms of fever and weight loss of two months’ duration. First, pneumonia was diagnosed but proved refractory to antimicrobial therapy. Computed tomography disclosed a large mediastinal mass compressing the left main bronchus with multiple bone metastases. Complete resection of the tumor was not feasible and a biopsy was performed by bronchoscopy. We detected the EWSR1-ATF1 fusion gene by RT-PCR and diagnosed the patient as having clear cell sarcoma. We initiated chemotherapy combined with local radiotherapy. Two weeks after the radiotherapy, the size of the primary tumor was reduced and the left main bronchus compression improved. We continued the chemotherapy and radiotherapy. However, the tumor increased in size two months after the completion of the radiotherapy, and the patient died of sepsis. In this case, radiotherapy was temporarily effective, but the development of a novel treatment is desirable.
