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中枢神经系统生殖细胞肿瘤:基于111例病例及其长期临床结果的治疗考量

Germ cell tumours of the central nervous system: treatment consideration based on 111 cases and their long-term clinical outcomes.

作者信息

Sawamura Y, Ikeda J, Shirato H, Tada M, Abe H

机构信息

Department of Neurosurgery, Hokkaido University School of Medicine, Sapporo, Japan.

出版信息

Eur J Cancer. 1998 Jan;34(1):104-10. doi: 10.1016/s0959-8049(97)10045-4.

Abstract

Germ cell tumours (GCTs) of the central nervous system (CNS) encompass various histological subtypes, and their optimal management has been the subject of debate. To indicate a better management strategy for each subtype, we analysed the records of 111 patients (median age 14 years), who underwent treatment since 1970. With a median follow-up duration of 86 months, the probability of surviving 5 years was: 96% for pure germinoma patients, 100% for mature teratoma, 67% for immature teratoma and 69% immature teratoma mixed with germinoma. The probability of cause-specific progression of germinomas producing human chorionic gonadotropin (HCG) was higher than that of non-producing germinomas (P < 0.01). GCTs that included a highly malignant component, such as embryonal carcinoma or yolk sac tumour, exhibited a poor prognosis with 38% chance of 5-year survival. Late adverse effects of therapy included stroke, secondary malignancy and cognitive, endocrinological, auditory and visual dysfunctions. Of 85 survivors with a median follow-up period of 99 months, 58 patients needed hormone replacement therapy, 26 patients showed poor performance status and, to date, only 1 patient has fathered children. Because the outcomes varied widely for each subtype, the traditional categories, that is, germinoma and non-germinomatous GCT as an extrapolation from the gonadal GCTs, are not suitable for appropriately selecting therapeutic regimen for CNS GCTs.

摘要

中枢神经系统(CNS)的生殖细胞肿瘤(GCTs)包含多种组织学亚型,其最佳治疗方案一直是争论的焦点。为了为每种亚型指明更好的治疗策略,我们分析了自1970年以来接受治疗的111例患者(中位年龄14岁)的记录。中位随访时间为86个月,5年生存率分别为:纯生殖细胞瘤患者96%,成熟畸胎瘤患者100%,未成熟畸胎瘤患者67%,未成熟畸胎瘤合并生殖细胞瘤患者69%。产生人绒毛膜促性腺激素(HCG)的生殖细胞瘤特异性病因进展概率高于不产生HCG的生殖细胞瘤(P<0.01)。包含高度恶性成分(如胚胎癌或卵黄囊瘤)的GCTs预后较差,5年生存率为38%。治疗的晚期不良反应包括中风、继发性恶性肿瘤以及认知、内分泌、听觉和视觉功能障碍。在85例中位随访期为99个月的幸存者中,58例患者需要激素替代治疗,26例患者表现不佳,迄今为止,只有1例患者育有子女。由于每种亚型的结果差异很大,传统分类,即从性腺GCTs推断而来的生殖细胞瘤和非生殖细胞瘤性GCT,不适用于为CNS GCTs适当选择治疗方案。

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