喉软骨肉瘤的长期气道后遗症和死亡风险。

文献信息

中文摘要

目的： 在低级别和中级别的喉软骨肉瘤中，非根治性手术甚至减瘤术被认为对于保留喉功能是合理的。然而，影响再次手术需求以及长期气管切开风险的相关肿瘤和治疗因素仍不清楚。
方法： 我们回顾性分析了2000年至2023年间在赫尔辛基大学医院（芬兰赫尔辛基）接受治疗的20例喉软骨肉瘤患者。
结果： 总共20例患者中有4例（20%）主要接受了全喉切除术。20例患者中有16例（80%）主要接受了开放部分喉切除术或内镜下减瘤术。在主要接受保喉手术治疗环状软骨肿瘤的患者中，因肿瘤复发或生长而再次手术（p = 0.003）以及从高分化或中分化组织学转化为低分化或去分化组织学（p = 0.027）与永久性气管切开显著相关。2例患者出现远处转移，2例死于喉软骨肉瘤。
结论： 主要表现为低级别或中级别软骨肉瘤的患者可能发生更高级别的局部复发和远处转移。由于喉软骨肉瘤的治疗范式已从根治性手术转向功能保留性手术，因此需要针对原发肿瘤评估和术后监测制定指南。为了最大限度地降低长期功能障碍和死亡风险，必须早期发现需要更积极治疗的不良肿瘤组织学或快速生长。

英文摘要

OBJECTIVE: In low- and intermediate-grade laryngeal chondrosarcomas, nonradical surgery or even debulking is considered justifiable to preserve laryngeal function. However, tumor- and treatment-related factors affecting the need for reoperation and long-term risk for tracheostomy remain unclear.
METHODS: We retrospectively analyzed 20 patients with laryngeal chondrosarcoma, treated at the Helsinki University Hospital (Helsinki, Finland) between 2000 and 2023.
RESULTS: In total, four of 20 (20%) patients primarily underwent total laryngectomy. Sixteen of 20 (80%) patients primarily underwent either open partial laryngectomy or endoscopic debulking. In patients with cricoid cartilage tumors primarily treated with larynx-preserving surgery, reoperation due to tumor recurrence or growth (p = 0.003) and transformation from well or moderately differentiated to poorly differentiated or dedifferentiated histology (p = 0.027) were significantly associated with permanent tracheostomy. Two patients presented with distant metastases and two died of laryngeal chondrosarcoma.
CONCLUSION: Patients primarily presenting with low- or intermediate-grade chondrosarcomas may develop higher-grade local recurrence and distant metastases. Due to paradigm shift from radical to function-preserving surgery in laryngeal chondrosarcoma, guidelines are needed for both primary tumor assessment and postoperative surveillance. To minimize the risk of long-term dysfunction and mortality, early detection of unfavorable tumor histology or rapid growth requiring more aggressive treatment is mandatory.