Fizazi K, Culine S, Droz J P, Le Chevalier T, Ruffié P, Théodore C
Département de médecine oncologique, Institut Gustave-Roussy, Villejuif, France.
Bull Cancer. 1997 Mar;84(3):313-27.
Primary mediastinal non-seminomatous germ-cell tumors (PMNSGCTs) are rare neoplasms that occur in young male adults. Incidence is evaluated about half that of extra-gonadal GCT. Their treatment is generally based on protocols used for testicular cancer, but with poorer results. Based on our experience of 40 patients with PMNSGCTs and data from the literature, we review here the clinical and biological data of these neoplasms. PMNSGCTs seem to constitute a specific entity, distinct from other GCT by the following criteria: true extra-gonadal origin, high incidence in patients with the Klinefelter's syndrome, over-representation of the yolk-sac component, poorer chemosensitivity and survival compared to other GCT, frequent occurrence of non-treatment related hematological neoplasia. The finding of an isochromosome of the short arm of the chromosome 12 in the leukemic karyotype is one of the strongest argument for a common origin in the yolk-sac component of the PMNSGCTs and their associated leukemia. Treatment of PMNSGCTs is still a challenge and should be conducted by a well-trained medical team.
原发性纵隔非精原细胞性生殖细胞肿瘤（PMNSGCTs）是发生于年轻成年男性的罕见肿瘤。其发病率约为性腺外生殖细胞肿瘤的一半。其治疗通常基于用于睾丸癌的方案，但效果较差。基于我们对40例PMNSGCTs患者的经验以及文献数据，我们在此回顾这些肿瘤的临床和生物学数据。PMNSGCTs似乎构成一个特定实体，根据以下标准与其他生殖细胞肿瘤不同：真正的性腺外起源、克兰费尔特综合征患者中的高发病率、卵黄囊成分的过度表现、与其他生殖细胞肿瘤相比化疗敏感性和生存率较差、非治疗相关血液肿瘤的频繁发生。在白血病核型中发现12号染色体短臂等臂染色体是PMNSGCTs及其相关白血病的卵黄囊成分存在共同起源的最有力证据之一。PMNSGCTs的治疗仍然是一项挑战，应由训练有素的医疗团队进行。
