Neurointervention Center, Beijing Tiantan Hospital, Capital Medical University, 100050 Beijing, China.
Department of Neurosurgery, Beijing, Chao-Yang Hospital, Capital Medical University, Beijing, China.
Neurochirurgie. 2021 Nov;67(6):587-598. doi: 10.1016/j.neuchi.2021.02.014. Epub 2021 Mar 19.
Primary intracranial pure endodermal sinus tumor (EST) is a rare malignant nongerminomatous germ cell tumor. There is still lack of knowledge about the treatment and prognosis.
We studied 6 cases of primary intracranial pure EST patients in Beijing Tiantan Hospital from June 2009 to June 2015. A comprehensive literature search was performed on Pubmed's electronic database using the search terms "primary intracranial endodermal sinus tumor", "primary intracranial yolk sac tumor" and "malignant germ cell tumor". Survival rates based on age, gender, tumor location, treatment, resection degree and metastatis were collected and analyzed.
Total resection was achieved in three patients and subtotal resection was achieved in three patients. Blood AFP level continuously decreased after tumor removal. In our case series, the longest survival time was 99 months; this patient received surgery+adjuvant therapy. Fifty-two cases were eligible and met the inclusion criteria for the review analysis. The majority of the population were in the young child to adolescent age and there is a male predominance in this study. In the Kaplan-Meier survival analysis, age, gender, tumor location and metastasis did not affect patients' OS. Better OS was found in patients who had subtotal and gross total resection of the tumor. Patients who had surgery combined with adjuvant therapy showed better OS compared to those who just had surgery.
Total resection of the tumor is necessary and EST is sensitive to adjuvant therapy.
原发性颅内纯内胚窦瘤（EST）是一种罕见的恶性非生殖细胞性生殖细胞瘤。目前对于其治疗和预后仍缺乏认识。
我们研究了 2009 年 6 月至 2015 年 6 月在北京天坛医院的 6 例原发性颅内纯 EST 患者。在 Pubmed 电子数据库中使用“primary intracranial endodermal sinus tumor”、“primary intracranial yolk sac tumor”和“malignant germ cell tumor”等检索词进行了全面的文献检索。收集并分析了基于年龄、性别、肿瘤位置、治疗、切除程度和转移的生存率。
3 例患者达到完全切除，3 例患者达到近全切除。肿瘤切除后，血 AFP 水平持续下降。在本病例系列中，最长生存时间为 99 个月；该患者接受了手术+辅助治疗。52 例符合纳入标准并符合综述分析条件。大多数患者为幼儿至青少年，本研究中男性居多。在 Kaplan-Meier 生存分析中，年龄、性别、肿瘤位置和转移均不影响患者的 OS。肿瘤行次全和大体全切除的患者 OS 更好。接受手术联合辅助治疗的患者 OS 优于仅接受手术的患者。
肿瘤的完全切除是必要的，EST 对辅助治疗敏感。
