纵隔脂肪肉瘤9例临床分析

Clinical analysis of 9 cases of mediastinal liposarcoma

目的结合9例纵隔脂肪肉瘤病历探讨纵隔脂肪肉瘤的临床特点和治疗。方法我院1970年～2007年共收治9例纵隔脂肪肉瘤患者,男性6例,女性3例,年龄36～63岁。临床均以胸闷、气短为主要表现,均接受过手术治疗,术后病理分型:5例为以粘液为主的脂肪肉瘤,2例为以梭型为主的脂肪肉瘤,2例为低度恶性脂肪肉瘤。其中4例患者接受放疗。结果死亡4例,其中3例死于局部复发。复发后再治疗3例,其中2例长期生存。结论脂肪肉瘤为恶性间叶性肿瘤中较常见的一种,多来源于退化的胸腺组织或纵隔胸膜脂肪组织,发生在纵隔极为罕见,占所有脂肪肉瘤的2.7%,不到所有纵隔肿瘤的1%。其病理学特点:主要成分为未成熟的脂肪细胞和成熟的脂肪细胞,以及纤维组织和性液组织。纵隔脂肪肉瘤的治疗应首选手术治疗,关键是尽早发现并及时彻底切除肿瘤。脂肪肉瘤对放疗和化疗的敏感性很低,复发和转移是较常见的失败原因。

Objective To explore the clinical features of mediastinal liposarcoma and its treatment. Methods Nine patients with mediastinal liposarcoma （6 men and 3 women; aged 36 -63 ） were treated in our hospital from 1970 to 2007. Chest distress and short breath were the common symptoms. All patients underwent operations. Postoperative pathologies showed lipesarcomas, including 5 with mucilage composition, 2 with spindle cell composition, and 2 with low potential malignancy. Four patients received radiotherapy. Results Four patients died, among whom 3 died of local recurrences. Local recurrences were found in 3 patients and 2 patients have long-term survival after retreatment. Conclusion Liposarcoma, a common malignant lobus intermedius tumor, mainly comes from degenerative thymus tissue or leipotissue of mediastinal pleura. Mediastinal liposarcoma is rare, with 1% in mediastinal tumors and 2. 7% in liposarcoma. The main compositions of mediastynal liposarcoma include immature leipoceU, mature leipocell, fibre tissue, and mucilage tissue. Surgery is the treatment of choice. Liposarcoma responds poor to radiotherapy and chemotherapy, with recurrence and metastasis as the common causes of treatment failure.