46例纵隔原发性生殖细胞肿瘤的临床病理学分析

Clinicopathological analysis of 46 cases of primary mediastinal germ cell tumors

目的探讨原发性纵隔生殖细胞肿瘤(PMGCTs)的临床病理学特点。方法收集46例PMGCTs患者的临床资料,对临床特点和病理学特征进行回顾性分析。结果 46例PMGCTs患者,男性24例,女性22例,平均年龄29.1岁。女性患者的病理学诊断均为成熟性畸胎瘤,恶性生殖细胞肿瘤的患者均为男性。PMGCTs患者的主要临床表现为胸痛、咳嗽、胸闷、气促、发热等。46例患者中,成熟性畸胎瘤34例(73.9%),精原细胞瘤4例(8.7%),卵黄囊瘤3例(6.5%),未成熟畸胎瘤2例(4.3%),混合性生殖细胞肿瘤2例(4.3%),恶性畸胎瘤1例(2.2%)。肿瘤组织免疫组织化学染色PLAP、OCT3/4、CD117、CK、CD30及LCA表达检测有助于诊断和鉴别诊断。随访发现,成熟性畸胎瘤和精原细胞瘤患者的5年生存率为分别为100%和75%,恶性畸胎瘤、卵黄囊瘤及混合性生殖细胞肿瘤患者的预后较差。结论 PMGCTs患者性别与肿瘤的组织学类型密切相关。成熟性畸胎瘤最常见,精原细胞瘤和卵黄囊瘤为常见的恶性生殖细胞肿瘤类型。与非精原细胞瘤性恶性生殖细胞肿瘤比较,精原细胞瘤患者预后较好,可能与其对放射治疗和化学治疗敏感有关。

Objective To investigate the clinicopathological features of primary mediastinal germ cell tumors （PMGCTs）. Methods A total of 46 patients with PMGGTs were selected and their clinicopathological features were retrospectively analyzed. Results Among 46 patients with PMGCTs, 24 of them were male and 22 of them were females and the median age was 29. 1. All 22 female patients were diagnosed with mature teratoma. All patients with malignant GCTs were male. Main clinical manifestations were chest pain, cough, dyspnea, and fever. Among 46 patients, there were 34 patients with mature teratoma （73.9%）, 4 patients with seminoma （8.7%）, 3 patients with york sac tumor （6.5%）, 2 patients with immature teratoma （4.3%）, 2 patients with mixed GCTs （4.3%）, and 1 patient with malignant teratoma （2.2%）. Immunohistochemical markers PLAP, OCT3/4, CD1l7, GK, GD30 and LGA were helpful for accurate diagnosis and differential diagnosis of PMGGTs. Follow-ups showed that the 5-year overall survival rates of padents with mature teratoma and seminoma were 100% and 75% , respectively. The prognosis of malignant teratoma, york sac tumor, and mixed GCTs was poor. Conclusion The gender of patients with PMGCTs is closely correlated with histological types of PMGCTs. Mature teratoma is the most common type. Seminoma and york sac tumor are common types of malignant GCTs. Compared with malignant GCTs other than seminoma, the prognosis of seminoma is much better, which may be relevant to being sensitive to radiotherapy and chemiotherapy.