原发性纵隔恶性生殖细胞肿瘤临床和预后分析

目的探讨原发性纵隔恶性生殖细胞肿瘤(PMGCT)的临床病理特点、治疗方法和预后因素。方法回顾性分析29例PMGCT患者的临床资料。结果29例患者均为男性,平均发病年龄26.1岁,肿瘤均来源于前纵隔,平均最大径16.0 cm。其中原发性纵隔精原细胞瘤(PMSGCT)5例(17.2%),原发性纵隔非精原细胞瘤(PMNSGCT)24例(82.8%)。PMGCT最常见症状是憋气、咳嗽与胸痛,其治疗采用化疗、手术、放疗相结合的综合治疗模式。PMNSGCT组中化生存期为19.0个月, 1年和2年生存率分别为65.3%和28.1%。PMSGCT组均长期生存,预后优于PMNSGCT组(P= 0.008)。多因素分析结果显示,病变局限于纵隔、以顺铂为基础的联合化疗是PMNSGCT患者预后的独立影响因素。结论PMGCT以PMNSGCT为主,主要治疗手段是以顺铂为基础的联合化疗。PMNSGCT预后明显差于PMSGCT,并与病变范围、化疗与否相关。

Clinical analysis of primary mediastinal germ cell tumors

OBJECTIVE: To investigate the clinical and pathological features, optimal treatment and prognostic factor in primary mediastinal germ cell tumors (PMGCT). METHODS: The clinical presentation, pathological features and therapeutic results of 29 patients with PMGCT were retrospectively analyzed. RESULTS: All the 29 patients were male with a mean age of 26.1 +/- 9.6 years at diagnosis. All tumors were originated from the anterior mediastinum with a mean diameter of 16.0 +/- 5.2 cm. There were 5 (17.2%) primary mediastinal seminomas (PMSGCT) and 24 (82.8%) primary mediastinal nonseminomas (PMNSGCT) in this series. Dyspnea, cough and chest pain were the most common symptoms. Multimodality treatment consisting of cisplatin-based chemotherapy, surgery and radiotherapy was the principal therapy for PMGCT patients. The median survival of the 24 PMNSGCT patients was 19.0 months with 1-, 2-year survival rate of 65.3% and 28.1%, respectively; whereas the median survival of the 5 PMSGCT patients has not reached but longer with significant differences (P = 0.008). Cox multivariate analysis indicated that limited mediastinal disease at diagnosis (P = 0.004) and the use of cisplatin-based combined chemotherapy (P = 0.005) were independent good prognostic factors of PMNSGCT. CONCLUSION: Primary mediastinal nonseminoma constitutes the most of primary mediastinal germ cell tumors. Cisplatin-based combined chemotherapy may be the most effective for the treatment of primary mediastinal germ cell tumors. The prognosis of primary mediastinal nonseminomas is significantly worse than that of primary mediastinal seminomas, and correlated with the extent of disease and chemotherapy.