ENT, Central Manchester University Hospitals NHS Foundation Trust, Manchester, Greater Manchester, UK
ENT, Central Manchester University Hospitals NHS Foundation Trust, Manchester, Greater Manchester, UK.
BMJ Case Rep. 2020 Oct 30;13(10):e237025. doi: 10.1136/bcr-2020-237025.
Embryonal rhabdomyosarcoma (ERMS) of the larynx in adults is an extremely rare diagnosis with insidious onset and progression. Only six reports (including this one) have been documented in the literature. Clinical presentation is dependent on the site, size, subtype of ERMS and growth rate. Hoarseness is the usual first symptom, followed by stridor and dyspnoea, with dysphagia being late in onset. Accurate staging and risk stratification is necessary to avoid overtreating/undertreating patients and should be guided by local Head and Neck/Sarcoma Multidisciplinary Teams. Treatment has moved away from radical therapeutic regimens to less-invasive, organ-preserving therapies. Long-term follow-up is required due to the risk of late recurrence.
成人喉胚胎性横纹肌肉瘤(ERMS)是一种极为罕见的诊断,其发病隐匿,进展缓慢。文献中仅记载了六例(包括本例)。临床表现取决于 ERMS 的部位、大小、亚型和生长速度。声音嘶哑是常见的首发症状,其次是喘鸣和呼吸困难,吞咽困难出现较晚。准确的分期和危险分层对于避免过度治疗/治疗不足的患者是必要的,应根据当地的头颈部/肉瘤多学科团队来指导。治疗已经从激进的治疗方案转向创伤更小、保留器官的治疗方法。由于存在晚期复发的风险,需要长期随访。