Saxby Clair, Bova Ron, Edwards Melanie
St Vincents Hospital, Sydney, NSW 2010, Australia.
Case Rep Otolaryngol. 2013;2013:578606. doi: 10.1155/2013/578606. Epub 2013 Jul 8.
Introduction. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells. Clinical Report. A 20-year-old male presented with an acute onset of respiratory stridor. Computer tomography scanning confirmed a mass arising from the left supraglottic larynx and an emergency tracheostomy was performed. A diagnosis of biphasic synovial sarcoma was formed. A total laryngectomy and left hemithyroidectomy was performed in conjunction with a left modified radical neck dissection. The patient received adjuvant chemotherapy followed by a course of radiotherapy and remains alive and disease free at 18 months after treatment. Discussion. Prognosis for patients with SS is related to primary tumour extent, grade, and size. The presence of the diagnostic translocation, t(X;18), is being targeted and hopefully will lead to the development of new therapeutics (Guadagnolo et al., 2007). Conclusion. Laryngeal SS remains a rare and poorly understood entity. A multidisciplinary approach to treatment is essential and long-term followup is imperative.
引言。滑膜肉瘤(SS)是侵袭性恶性软组织肿瘤,被认为起源于多能间充质细胞。
临床报告。一名20岁男性突发呼吸喘鸣。计算机断层扫描证实肿块起源于左声门上喉,遂行紧急气管切开术。诊断为双相滑膜肉瘤。行全喉切除术、左半甲状腺切除术并结合左改良根治性颈清扫术。患者接受辅助化疗,随后进行一个疗程的放疗,治疗后18个月仍存活且无疾病。
讨论。SS患者的预后与原发肿瘤范围、分级和大小有关。诊断性易位t(X;18)的存在正成为治疗靶点,有望带来新疗法的开发(瓜达尼奥洛等人,2007年)。
结论。喉SS仍然是一种罕见且了解甚少的疾病。多学科治疗方法至关重要,长期随访势在必行。