成年人的舌头胚胎性拉布多米索尔科马
Alberto Díez-Montiel1, Raúl Antúnez-Conde1, Carlos Navarro Cuéllar1
1Department of Oral and Maxillofacial Surgery, Instituto de Investigación Sanitaria Gregorio Marañon (liSGM), Gregorio Marañon General University Hospital, 28007 Madrid, Spain.
Life (Basel, Switzerland)
|June 28, 2023
概括
这份病例报告强调了一种极为罕见的成年人舌肌肉瘤. 成功的治疗包括手术和化疗,导致无疾病的结果,并保留了舌头功能.
科学领域:
- 在瘤学瘤学.
- 手术病理学手术病理学
- 医疗成像医学成像
背景情况:
- 狂肌肉瘤 (RMS) 是一种罕见的软组织肉瘤,主要影响儿童和青少年.
- 头部RMS占儿科病例的三分之一,胚胎类型是最常见的.
- 成年RMS非常罕见,仅占成年恶性瘤的1%.
研究的目的:
- 报告一种罕见的成年胚胎舌肉瘤 (RMS) 病例.
- 讨论成年人这种罕见实体的诊断和治疗挑战.
- 强调完整的外科切除和辅助化疗的重要性.
主要方法:
- 一名46岁的男性出现了逐渐增长的舌头损伤.
- 通过切除活检和免疫组织化学 (INI-1阳性,FOXO1A/MDM2阴性) 确认了诊断.
- 用对比度增强的MRI用于病变的表征和手术规划.
主要成果:
- 患者接受了局部片重建的部分光切除术.
- 辅助化疗包括八个周期的VAC (温克里斯,阿克丁诺米辛,环胺) 协议.
- 患者在治疗后42个月实现了无病状态,功能恢复良好.
结论:
- 胚胎性舌头RMS在成年人中非常罕见,文献先例有限.
- 与儿科患者相比,患有RMS的成年人通常预后较差.
- 多种模式的治疗,包括完整的外科切除和适当的化疗,对于有利的结果至关重要.
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