脂肪肉瘤,恶性脂肪细胞肿瘤,主要表现在下肢和腹膜后,在头部和颈部区域的发病率非常低。症状学通常不存在,直到肿瘤达到显著的大小,导致美容问题或压迫相关并发症。本报告介绍了一名81岁男性的高分化咽后脂肪肉瘤的独特病例,强调诊断挑战,管理策略,和小鼠双分2(MDM2)荧光原位杂交(FISH)分析在确认中的关键作用。患者表现出吞咽困难,归因于咽后肿块,引发对恶性肿瘤的怀疑.诊断评估,包括柔性喉镜和经皮穿刺活检,突出了独特的特征,如脂肪细胞的非典型核特征。MDM2FISH分析通过检测MDM2基因扩增明确证实了诊断。咽后脂肪肉瘤的罕见性使诊断复杂化,常导致与良性病变混淆。手术切除,治疗的主体,根据肿瘤大小和延伸而变化。在这种情况下,通过曲棍球棒切口进行的左颈淋巴结清扫术成功切除了一个17厘米高分化的脂肪肉瘤。病理分析显示切除边缘局部受累,无并发症或声带损伤。总之,咽后脂肪肉瘤构成诊断挑战,保证依赖MDM2FISH分析进行准确确认。早期手术干预,以肿瘤大小和延伸为导向,对于管理这些罕见肿瘤的最佳结果至关重要。此病例强调了详细的手术方法在咽后脂肪肉瘤的成功治疗中的重要性。
Liposarcomas, malignant adipocytic tumors, primarily manifest in the lower extremities and retroperitoneum, with a strikingly low incidence in the head and neck region. Symptomatology typically remains absent until the tumor attains significant size, leading to cosmetic concerns or compression-related complications. This report presents a unique case of well-differentiated
retropharyngeal liposarcoma in an 81-year-old male, emphasizing diagnostic challenges, management strategies, and the crucial role of Mouse double minute 2 (MDM2) fluorescence in situ hybridization (FISH) analysis in confirmation. The patient exhibited dysphagia attributed to a
retropharyngeal mass, prompting suspicion of malignancy. Diagnostic assessments, including flexible laryngoscopy and percutaneous tru-cut biopsy, highlighted unique features such as atypical nuclear features in adipocytes. MDM2 FISH analysis definitively confirmed the diagnosis by detecting MDM2 gene amplification. The rarity of
retropharyngeal liposarcomas complicates diagnosis, often leading to confusion with benign lesions. Surgical excision, the mainstay of treatment, varies based on tumor size and extension. In this case, a left neck dissection via a hockey stick incision successfully resected a 17 cm well-differentiated liposarcoma. Pathologic analysis revealed focal involvement of resection margins, with no complications or vocal cord damage. In conclusion,
retropharyngeal liposarcomas pose diagnostic challenges, warranting reliance on MDM2 FISH analysis for accurate confirmation. Early surgical intervention, guided by tumor size and extension, is paramount for optimal outcomes in managing these rare tumors. This case underscores the significance of a detailed surgical approach in achieving successful outcomes for
retropharyngeal liposarcomas.