Department of Oncology, Aga Khan University, Karachi,Pakistan.
Department of Cyberknife Robotic Radiosurgery, Jinnah Postgraduate Medical Centre, Karachi, Pakistan.
J Pak Med Assoc. 2023 Jun;73(6):1297-1301. doi: 10.47391/JPMA.6193.
Synovial Sarcoma (SS) is a rare soft-tissue malignant tumour. Its presentation in the head and neck region is uncommon. Because of the complex anatomy of the head and neck region, surgery with clear margins is not achievable. In such cases, a multi-modality approach is required as there is no established standard of care. In this report, we share the case of a girl who presented with nasal obstruction. Imaging revealed a mass involving the left nasal cavity, paranasal sinuses without intracranial extension. It was diagnosed as synovial sarcoma. She underwent surgical excision and adjuvant radiation therapy (RT) to the tumour bed, followed by an incomplete course of chemotherapy. Later on, she developed systemic disease. Considering the rarity of this case and lack of guidelines for standard treatment, we report on this case to share our experience with management and treatment outcome.
滑膜肉瘤（SS）是一种罕见的软组织恶性肿瘤。其在头颈部的表现并不常见。由于头颈部的解剖结构复杂，无法实现明确切缘的手术。在这种情况下，由于没有既定的治疗标准，需要采用多模态方法。在本报告中，我们分享了一个女孩的病例，她因鼻塞就诊。影像学显示左侧鼻腔、鼻窦有肿块，无颅内扩展。诊断为滑膜肉瘤。她接受了手术切除和肿瘤床辅助放疗（RT），随后进行了不完全疗程的化疗。后来，她出现了全身疾病。考虑到这种情况罕见，且缺乏标准治疗的指南，我们报告这个病例以分享我们的管理和治疗结果的经验。
