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目的原发性胸膜脂肪肉瘤是一种非常罕见的肿瘤,本文报道国内首例病例,并探讨其临床特点、诊断及治疗。方法分析我院近期发现的1例原发性胸膜脂肪肉瘤的临床资料并复习相关文献。结果原发性胸膜脂肪肉瘤全世界仅报道17例(包括此例)。本病好发于男性,中老年人多见,病理类型以黏液型多见;临床症状以胸痛和气促最常见;诊断主要依靠影像学和开胸探查。目前治疗推荐手术切除加辅助放疗。结论原发性胸膜脂肪肉瘤的治疗和预后的评估有待进一步积累资料。
Objective Primary pleuriposarcoma is a very rare tumor, this article reports the first case in China, and discusses its clinical features, diagnosis and treatment. Methods One case of primary pleural liposarcoma discovered in our hospital was analyzed and the related literatures were reviewed. Results Primary pleural liposarcoma worldwide only reported 17 cases (including this case). The disease occurs in men, more common in older adults, more common pathological type to mucus; clinical symptoms of chest pain and shortness of breath are the most common; diagnosis depends mainly on imaging and open chest exploration. The current treatment recommended surgery plus adjuvant radiotherapy. Conclusion The evaluation of the treatment and prognosis of primary pleural liposarcoma needs to be further accumulated.