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报道性腺和性腺外内胚窦瘤(EST)56例,其中发生于尿道口二例及耻骨后膀胱前一例,文献未见报道。结合文献资料1245例分析,性腺外EST发生部位为身体中线和近中线部位。EST发病年龄大多为婴儿、儿童和35岁以下的青年。一些性腺外EST有部位、年龄、性别的特点。如纵隔EST几全发生在男性青年(迄今仅有一例为两个月女婴),而骶尾部EST则多为婴幼儿,女性居多。松果体EST多为儿童,男多于女。病理上可分为单纯型EST和与其他性腺肿瘤成分混合的混合性性腺细胞EST(MGCEST)型。其组织结构有特征性,确诊不难。加上血清甲胎球蛋白(AFP)值测定,可监测手术、治疗效果、复发和转移情况。EST发展迅速,为高度恶性肿瘤。不论单纯型或MGCEST型(后者成分可多可少),预后极差。因此建议凡畸胎瘤病例,治疗前后应进行血清AFP测定。对纯精原(或无性)细胞瘤与EST成分混合病例,测定血清AFP值可以证明文献上学者的两种意见。一种意见认为纯精原(或无性)细胞瘤与EST成分混合的MGCEST型,预后比单纯型EST和混合有性腺瘤其他成分的MGCEST好。另一种意见则相反。睾丸EST婴幼儿组较青年组预后好。性腺外EST预后较性腺EST差。但文献资料阴道EST预后较好。近年来对EST进行手术加(或)联合化疗,或先联合化疗后手术,加上测定血清AFP监测,使一些病例能延长寿命。
Reported 56 cases of gonadal and extra-glandular endodermal sinus tumor (EST), of which occurred in the urethra in two cases and the retropubic bladder before a case, the literature has not been reported. Combined with 1245 cases of literature data, extragonadal ESTs occurred in the midline and near midline. EST age of onset mostly infants, children and youth under 35 years of age. Some extragonadal ESTs have parts, age, gender characteristics. For example, mediastinal ESTs occur mostly in young males (to date, only one case is a two-month old baby girl), while sacrococcygeal ESTs are mostly infants and young females. Pineal EST mostly children, more men than women. Pathological can be divided into simple EST and mixed with other gonadal components of mixed gland cell EST (MGCEST) type. Its organizational structure is characteristic, diagnosis is not difficult. Together with serum AFP values, surgeries, treatment effects, relapse and metastasis can be monitored. EST develops rapidly and is a highly malignant tumor. Whether simple or MGCEST type (the latter can be more or less less), the prognosis is poor. Therefore, the proposed teratoma cases, before and after treatment should be serum AFP determination. Mixed samples of pure spermatogonia (or asexual) cell tumor and EST, determination of serum AFP values can prove two opinions of scholars in the literature. One opinion is that MGCEST, a mixture of pure spermatogonia (or anaplastic) cell tumor and EST, has a better prognosis than MGCEST, which is a combination of simple EST and other components of the adenoma. The other is the opposite. Testicular EST infants better prognosis than young group. Prostate extrahepatic EST prognosis worse than the gonadal EST. However, the literature Vaginal EST prognosis is good. In recent years, EST plus surgery (or) combined with chemotherapy, or combined with chemotherapy before surgery, coupled with the determination of serum AFP monitoring, so that some cases can extend life expectancy.