Zucker J M
Bull Cancer. 1982;69(3):304-13.
Malignant ovarian tumors only account for 3 per cent of all malignancies under 15 years of age, and more than two third of them are of germ cell origin. Abdominal mass and/or pains are the usual revealing symptoms and abnormal sexual prematurity is infrequently encountered. Diagnostic ultrasonography is currently the most useful imaging investigation. Alpha-foeto-protein (alpha-FP) serum determination is mandatory before surgery to indicate the presence of endodermal sinus tumor cells. Surgical procedure is in childhood usually restricted to unilateral salpingo-oophorectomy, if allowed by tumoral spreading which is carefully searched in the abdominal cavity. Thus will be defined, along with the post-operative lymphangiography, the extension of the tumor, according to the staging system of Wollner (Memorial Hospital NY) more than the FIGO. Malignant teratomas are the most frequent malignant germ cell ovarian tumors in young females. They may realize pure or mixed pathological types of immature teratoma, embryonal carcinoma, endodermal sinus tumor and very seldom choriocarcinoma. Serial assays of serum alpha-FP are of utmost value to follow therapeutic progress or detect recurrences. Since new sequential multidrug protocol including, vincristine, actinomycin D, cyclophosphamide, adriamycin, méthotrexate and cis-platinum, protracted survivals are to be expected in a higher number of patients with localized stages and even in some with advanced disease. Value of extended pelvi-abdominal and/or lymph node radiotherapy is still under evaluation. Ovarian dysgerminomas are radiosensitive tumors in which chemotherapy is as a rule not indicated. Relapses may be most often prevented by a prophylactic irradiation of iliac ipsilateral, lumboaortic, mediastinal and supraclavicular nodes. Among functional ovarian tumors, mainly granulosa cell types with isosexual precocity, malignant tumors are so rarely encountered that no complementary treatment has to be performed after a complete resection. Cystadenocarcinoma or borderline ovarian epithelial tumors occur after puberty and require the same treatment as in adult though keeping a conservative view. Ovarian deposits of Burkitt's tumor may account in some exposed african areas for most ovarians malignancies in childhood. Gonadoblastomas with both stromal and germ cell elements are exclusively to be found in dysgenetic gonads.
恶性卵巢肿瘤仅占15岁以下所有恶性肿瘤的3%，其中超过三分之二起源于生殖细胞。腹部肿块和/或疼痛是常见的首发症状，性早熟异常并不常见。诊断性超声检查是目前最有用的影像学检查。术前必须测定血清甲胎蛋白（α-FP）以提示内胚窦瘤细胞的存在。在儿童期，如果肿瘤在腹腔内的扩散情况允许（需仔细探查），手术通常限于单侧输卵管卵巢切除术。这样，结合术后淋巴管造影，根据沃尔纳（纽约纪念医院）分期系统而非国际妇产科联合会（FIGO）分期系统来确定肿瘤的范围。恶性畸胎瘤是年轻女性中最常见的恶性生殖细胞卵巢肿瘤。它们可能呈现纯型或混合型的未成熟畸胎瘤、胚胎癌、内胚窦瘤，绒癌则极为罕见。血清α-FP的系列检测对于追踪治疗进展或检测复发至关重要。自从采用包括长春新碱、放线菌素D、环磷酰胺、阿霉素、甲氨蝶呤和顺铂的新序贯多药方案后，预计更多局限性期患者甚至一些晚期疾病患者的生存期会延长。扩大盆腔腹部和/或淋巴结放疗的价值仍在评估中。卵巢无性细胞瘤是对放疗敏感的肿瘤，通常不进行化疗。通过对同侧髂骨、腰主动脉、纵隔和锁骨上淋巴结进行预防性照射，大多可预防复发。在功能性卵巢肿瘤中，主要是具有同性性早熟的颗粒细胞类型，恶性肿瘤极为罕见，完整切除后无需进行补充治疗。囊腺癌或卵巢交界性上皮肿瘤在青春期后发生，尽管秉持保守观点，但治疗方法与成人相同。在一些非洲暴露地区，伯基特淋巴瘤的卵巢浸润可能是儿童期大多数卵巢恶性肿瘤的原因。兼具基质和生殖细胞成分的性腺母细胞瘤仅见于发育异常的性腺中。
