食管上段胎儿横纹肌瘤:4 名儿童的病例系列及文献综述。

Zhiyong Liu, Yiran Meng, Yanzhen Li, Xuexi Zhang, Qiaoyin Liu, Nian Sun, Tingting Ji, Xiaodan Li, Yuwei Liu, Shengcai Wang, Xin Ni
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引用次数: 0

摘要

研究目的本研究旨在为鉴别儿童食管上段胎儿横纹肌瘤(RM)提供客观证据,提高临床对该疾病诊断和治疗的认识,优化胎儿横纹肌瘤的治疗策略。方法:回顾性收集2016年7月至2022年7月期间我院耳鼻咽喉头颈外科收治的4例食管上段胎儿RM患儿的临床病历。结合文献分析了他们的临床、组织学和治疗特点。结果:共纳入4例确诊为食管上段肿瘤的患儿,所有患儿均接受了食管上段肿瘤切除+食管咽部重建术,其中2例患儿因喉返神经粘连接受了预防性气管切开术。其中 2 例(病例 2 和病例 4)进行了术前活检,另外 2 例(病例 1 和病例 3)进行了术中冰冻切片分析,病理结果与胎儿 RM 一致。术后对患者进行了 25 至 96 个月的随访。到目前为止,只有 1 例患者的胎儿 RM 复发,并接受了第二次手术切除肿瘤。结论:胎儿 RM 是一种良性肿瘤:胎儿 RM 是一种易复发的良性肿瘤,完全切除是首选的最佳治疗方法。临床医生需要了解并掌握胎儿 RM 的管理算法,以规范其诊断和治疗。
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Upper Esophageal Fetal Rhabdomyoma: A Case Series of 4 Children and Review of Literature.

Objective: The aims of the present study were to provide objective evidence for identifying fetal rhabdomyoma (RM) in the upper esophagus of children, enhance clinical understanding of the diagnosis and treatment of this condition, and optimize the treatment strategy for fetal RM. Methods: The clinical medical records of 4 children with upper esophageal fetal RM were retrospectively collected, and were admitted to the Department of Otolaryngology, Head and Neck Surgery at our hospital between July 2016 and July 2022. Their clinical, histological, and therapeutic characteristics were analyzed in combination with the literature. Results: Four children diagnosed with upper esophageal tumors were included and all of them underwent resection of the upper esophageal tumor with esophageal-pharyngeal reconstruction, and 2 of them underwent prophylactic tracheotomy due to recurrent laryngeal nerve adhesion. Preoperative biopsy was performed in 2 cases (case 2 and case 4), while intraoperative frozen section analysis was conducted in the other 2 (case 1 and case 3), with pathological results consistent with fetal RM. Patients were followed up for 25 to 96 months after the surgery. So far, only 1 patient has experienced a recurrence of fetal RM and underwent a second surgical resection to remove the tumor. Conclusion: Fetal RM is a benign tumor prone to recurrence, and complete excision is the preferred optimal treatment. Clinicians need to understand and master the management algorithm for fetal RM to standardize its diagnosis and treatment.

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