Lemarie E, Lavandier M, Renjard L, Allard P, Negra J P, Toumieux B
Service de Pneumologie, CHU Bretonneau, Tours.
Rev Mal Respir. 1989;6(2):95-108.
The primary malignant dysembryomas of the mediastinum are highly malignant tumours, which originate from embryonal cells. This name excludes seminomas and dysgerminomas which derive from non-fecund germinal cells. In this general review only the dysembryomas will be touched on. They are relatively rare tumours found in young men and are characterised by the rapidity of their spontaneous growth. The absence of a gonadal tumour is sufficient to confirm the primary mediastinal character of malignant dysembryomas. There is little specific in the clinical data. The tumour markers, Beta HCG and above all alpha feto-protein play, at the same time, a leading role not only for diagnosis but also for therapeutic decisions and follow up under treatment. In spite of the appalling reputed prognosis of this disease, the malignant dysembryomas have benefited from the considerable therapeutic progress which has been made in recent years. It is vital to achieve a rapid and accurate diagnosis and to start on treatment without delay with a regime incorporating Cisplatin. As radical surgery as possible usually follows the initial chemotherapy. Post operative treatment depends on the anatomical observations made at the time of surgery.
纵隔原发性恶性胚胎瘤是高度恶性肿瘤,起源于胚胎细胞。这个名称不包括源自未受精生殖细胞的精原细胞瘤和无性细胞瘤。在这篇综述中,仅涉及胚胎瘤。它们是相对罕见的肿瘤,多见于年轻男性,其特点是自发生长迅速。无性腺肿瘤足以证实恶性胚胎瘤的原发性纵隔特征。临床资料特异性不强。肿瘤标志物β-HCG尤其是甲胎蛋白,不仅在诊断中起主导作用,而且在治疗决策和治疗过程中的随访中也起主导作用。尽管这种疾病的预后声名狼藉,但恶性胚胎瘤受益于近年来取得的显著治疗进展。快速准确地诊断并立即开始采用含顺铂的方案进行治疗至关重要。初始化疗后通常尽可能进行根治性手术。术后治疗取决于手术时的解剖观察结果。