Germ cell tumours form an important group of gonadal neoplasms and are also found in a number of extragonadal sites like the mediastinum, para-pineal and sacrococcygeal regions and retroperitoneum. Although there are considerable differences between germ cell tumours occurring in different anatomical locations they exhibit a remarkable homology, and are considered as a group. In this review germ cell tumours are discussed as a group emphasizing some of the recent developments in this field. In the testis germ cell tumours form the most common group of neoplasms comprising 90% of all testicular tumours and 99% of them are malignant. In the ovary germ cell tumours comprise approximately 20% of ovarian neoplasms, and more than 90% are mature cystic teratomas and are benign. Malignant testicular neoplasms are 10 times more common than their ovarian and 20 times more common than their extragonadal counterparts. Malignant germ cell tumours have a specific age incidence and occur mainly in children and young adults. Due to this they represent one of the most important groups of neoplasms in this age group. Testicular germ cell tumours show marked racial and geographical differences occurring much more frequently in Western Europe, especially in Scandinavia, as compared with Southern and Eastern Europe. They are rare in Africa and are very uncommon in Blacks as compared to Whites. These remarkable differences are not observed in ovarian or extragonadal germ cell tumours. It is now accepted that histogenetically all the tumours in this group are of germ cell origin, and that germ cell tumours are capable of somatic (embryonal) and extra-embryonal differentiation (fig. 1). The occurrence of extragonadal germ cell tumours in anatomical locations in the midline of the body is explained on the basis of migration of the primitive germ cells during embryonic life from the wall of the yolk sac to the primitive gonad. An all embracing classification of germ cell neoplasms based on the WHO classifications of ovarian and testicular tumours is presented. The importance of careful and thorough examination of germ cell tumours is emphasized, especially in view of the recent advances in the therapy of malignant germ cell neoplasms. The value of tumour markers like alphafoetoprotein (AFP) and human chorionic gonadotropin (HCG) produced by endodermal sinus tumour (EST) and some embryonal carcinomas and choriocarcinoma and syncytiotrophoblastic giant cells respectively in diagnosis, monitoring the progress of the disease, and the efficacy of therapy, as well, as for early detection of metastases and recurrences, is strongly emphasized.(ABSTRACT TRUNCATED AT 400 WORDS)
生殖细胞肿瘤是性腺肿瘤中的一个重要类别,也见于许多性腺外部位,如纵隔、松果体旁、骶尾部及腹膜后区域。尽管发生于不同解剖部位的生殖细胞肿瘤存在显著差异,但它们表现出明显的同源性,因此被视为一个群体。在本综述中,将把生殖细胞肿瘤作为一个群体进行讨论,重点介绍该领域的一些最新进展。在睾丸,生殖细胞肿瘤是最常见的肿瘤类型,占所有睾丸肿瘤的90%,其中99%为恶性。在卵巢,生殖细胞肿瘤约占卵巢肿瘤的20%,其中90%以上是成熟囊性畸胎瘤,为良性。恶性睾丸肿瘤比卵巢恶性生殖细胞肿瘤常见10倍,比性腺外恶性生殖细胞肿瘤常见20倍。恶性生殖细胞肿瘤有特定的年龄发病率,主要发生于儿童和青年。因此,它们是该年龄组中最重要的肿瘤群体之一。睾丸生殖细胞肿瘤表现出明显的种族和地理差异,与南欧和东欧相比,在西欧尤其是斯堪的纳维亚半岛更为常见。在非洲罕见,与白人相比,在黑人中非常少见。卵巢或性腺外生殖细胞肿瘤则未观察到这些显著差异。现在已公认,从组织发生学角度来看,该组所有肿瘤均起源于生殖细胞,并且生殖细胞肿瘤能够进行体细胞(胚胎性)和胚外分化(图1)。身体中线解剖部位出现性腺外生殖细胞肿瘤,是基于胚胎期原始生殖细胞从卵黄囊壁迁移至原始性腺来解释的。本文根据世界卫生组织对卵巢和睾丸肿瘤的分类,给出了一个全面的生殖细胞肿瘤分类。强调了对生殖细胞肿瘤进行仔细和全面检查的重要性,特别是鉴于恶性生殖细胞肿瘤治疗方面的最新进展。还强烈强调了肿瘤标志物的价值,如内胚窦瘤(EST)以及一些胚胎癌、绒毛膜癌和合体滋养层巨细胞分别产生的甲胎蛋白(AFP)和人绒毛膜促性腺激素(HCG),在诊断、监测疾病进展、评估治疗效果以及早期发现转移和复发方面的作用。(摘要截选至400字)