A Case of a Cervical Esophageal Synovial Sarcoma Treated with Proton Beam Therapy Enabling Larynx-preserving Cervical Esophagectomy

Abstract

The patient was a 22-year-old woman who presented with dysphagia. Imaging and endoscopic evaluation showed a 13-cm esophageal tumor occupying the cervical and upper thoracic esophagus. Further workup confirmed a diagnosis of esophageal synovial sarcoma positive for the SS18-SSX1 fusion gene. The standard treatment for synovial sarcoma is wide resection with adequate surgical margins ; in this case, tumor resection would have necessitated total laryngectomy and subtotal esophagectomy. To preserve laryngeal function, however, definitive radiotherapy was selected. Proton beam therapy was chosen to enhance local tumor control while minimizing radiation-related toxicities by reducing the dose to surrounding normal tissues. Following proton beam therapy, its size was decreased, and it became localized to the cervical esophagus. This allowed for preservation of the larynx and avoidance of subtotal esophagectomy, enabling cervical esophagectomy to be performed instead. At present, she remains alive without recurrence 3 years and 6 months after surgery.