19例成人原发性纵隔脂肪肉瘤临床分析

摘要

Objective To explore the pathoclinical features of adult primary mediastinal liposarcoma and their correlation with the prognosis. Methods The clinical data of 19 patients with adult primary mediastinal liposarcoma who were treated in our hospital between 1970 and 2011 were retrospectively analyzed. Eighteen patients underwent open thoracic tumor excisions for at least one time, and the remaining one patient only received biopsy surgery. Histopathological results after surgery revealed that 6 well-differentiated type tumors, 6 myxoid type tumors, 3 pleomorphic type tumors, 2 mixed type tumors, and 2 un-classified tumors. Results Among 6 patients with well-differentiated type tumors, 1 died after having been survived for 10 years; 5 were still alive, with a mean duration of 126. 2 months, the 5-year survival rate was 100%. Among 6 patients with myxoid type tumors, 5 patients had follow-up data, with a mean survival of 26. 2 months. Among 3 patients with pleomorphic type tumors, only one patient had follow-up data: the patient finally died, with a survival of 34 months. Of 2 patients with mixed type tumors, only one patient had follow-up data: the patient survived 8 months and died. Of 2 patients with un-classified type tumors, one had follow-up data: the patient lived for 24 months and died. Conclusions Mediastinal liposarcoma is a rare disease. Surgery is the primary therapeutic modality. Different pathological subtypes have different epidemiological features, biological behaviors, and ma-lignant potentials. Pathological subtype is an important prognosis factor. Patients with well-differentiated tumors have much better prognosis than those with other subtypes.%目的 探讨成人原发性纵隔脂肪肉瘤的临床和病理特点及其与预后的关系.方法 回顾性分析了19例成人原发性纵隔脂肪肉瘤患者的临床资料,其中,18例患者至少接受过1次开胸肿物切除手术,1例只接受活检手术；6例为高分化脂肪肉瘤,6例为黏液样脂肪肉瘤,3例为多形性脂肪肉瘤,2例为混合型脂肪肉瘤,2例不能分类.结果 6例高分化脂肪肉瘤患者中,死亡1例,生存期10年；生存5例,至今平均生存126.2个月,5年生存率100％.6例黏液样脂肪肉瘤患者中,失访1例,死亡5例,平均生存26.2个月.3例多形性脂肪肉瘤患者,失访2例,死亡1例,生存期34个月.2例混合型脂肪肉瘤患者,失访1例,死亡1例,生存期8个月.未能进行病理亚分型的2例中,失访1例,死亡1例,生存期24个月.结论 成人原发性纵隔脂肪肉瘤十分罕见,手术是其主要治疗方式.不同病理亚型的流行病学特点、生物学行为、恶性潜能各有不同,是影响预后的重要因素.高分化脂肪肉瘤预后明显好于其他亚型.