Burt M, Ihde J K, Hajdu S I, Smith J W, Bains M S, Downey R, Martini N, Rusch V W, Ginsberg R J
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
J Thorac Cardiovasc Surg. 1998 Mar;115(3):671-80. doi: 10.1016/S0022-5223(98)70333-2.
Primary sarcomas of the mediastinum are rare, and data concerning treatment and results of therapy are sparse.
To assess presentation, management, prognostic factors, and survival in mediastinal sarcomas.
We reviewed our experience with 47 patients with the diagnosis of primary sarcoma of the mediastinum. Data were collected from a computerized institutional database and medical records. Survival was analyzed by Kaplan-Meier method and comparisons of survival by log rank test.
The median age of 47 patients with mediastinal sarcoma was 39 years (range 2.5 to 69 years), with a male/female ratio of 1.6. The most common complaints were chest/shoulder pain (38%) and dyspnea (23%). The most common tumor types were malignant peripheral nerve tumor (26%), spindle cell sarcoma (15%), leiomyosarcoma (9%), and liposarcoma (9%). Operation was the primary treatment modality in 72% of cases (n = 34); 22 sarcomas (47%) were completely resected. The overall 5-year survival was 32%. High-grade lesions had a significantly decreased survival (5-year survival = 27%) compared with low-grade tumors (5-year survival = 66%) (p = 0.05). The overwhelming factor determining survival was the ability to completely resect the tumors (5-year survival 49% for complete resection; 3-year survival 18% for incomplete or no resection) (p = 0.0016). Despite complete resection, local recurrence occurred in 64% of cases.
Because the overall survival for patients with mediastinal sarcomas is 32% and the local recurrence is 64% for tumors completely resected, aggressive adjuvant therapy should continue to be systematically explored.
纵隔原发性肉瘤罕见，有关治疗及治疗结果的数据稀少。
评估纵隔肉瘤的临床表现、治疗、预后因素及生存率。
我们回顾了47例诊断为纵隔原发性肉瘤患者的治疗经验。数据从计算机化的机构数据库和病历中收集。采用Kaplan-Meier法分析生存率，并通过对数秩检验比较生存率。
47例纵隔肉瘤患者的中位年龄为39岁（范围2.5至69岁），男女比例为1.6。最常见的症状是胸痛/肩痛（38%）和呼吸困难（23%）。最常见的肿瘤类型是恶性外周神经肿瘤（26%）、梭形细胞肉瘤（15%）、平滑肌肉瘤（9%）和脂肪肉瘤（9%）。72%的病例（n = 34）主要治疗方式为手术；22例肉瘤（47%）完全切除。总体5年生存率为32%。与低级别肿瘤（5年生存率 = 66%）相比，高级别病变的生存率显著降低（5年生存率 = 27%）（p = 0.05）。决定生存率的首要因素是完全切除肿瘤的能力（完全切除的5年生存率为49%；不完全切除或未切除的3年生存率为18%）（p = 0.0016）。尽管完全切除，64%的病例仍发生局部复发。
由于纵隔肉瘤患者的总体生存率为32%，完全切除的肿瘤局部复发率为64%，应继续系统地探索积极的辅助治疗。
