Tonsillar synovial sarcoma is a rare presentation of tumours in ENT, with only 19 well-documented cases reported in English literature. However, it is an aggressive malignancy with high potential for metastasis and recurrence. If diagnosed late, metastatic disease presents with drastically poorer 5-year survival rate compared to localized disease. This, alongside synovial sarcoma most affecting the demographic of children and young adults, highlights the importance of early clinical suspicion, accurate diagnosis, and effective evidence-based management. We present this case of a previously fit and well 26 year-old male who presented with right tonsillar enlargement, dysphonia, and halitosis. Pathological immunohistochemistry confirmed tonsillar synovial sarcoma. Pre-operative management involved chemotherapy (doxorubicin and ifosfamide) and radiotherapy. Surgical resection with wide negative margins is the gold standard for management, and the extensive operative procedure involved right oropharyngectomy, lip split, mandibulotomy, neck dissection, tracheostomy, and left radial forearm free flap reconstruction. Intraoperative real-time pathology aided surgical management. Complete margins could not be achieved due to the anatomical complexity related to the tumour, and the patient continued to complete 6 cycles of post-operative trabectedin. We present this case as an extensive multidisciplinary effort between ENT, plastics, and maxillofacial surgeons, alongside pathology and clinical and medical oncology. We also discuss utilizing the latest research in management despite the rarity of tonsillar synovial sarcoma, highlighting it as an essential differential when considering ENT pathologies.

Dr. Lun Zhu graduated in Medicine with the Rosemary Murray Scholarship and First Class Honours from the University of Cambridge, United Kingdom. She intercalated in an additional degree in Pathology (Immunology, Cancer, and Genetics) and Applied Clinical Research. She is currently working in her first foundation year as a doctor on the Isle of Wight St. Mary’s Hospital, and has an interest in policy and management as well as clinical teaching.