T. Y. Nelson Department of Neurology and Neurosurgery, The Children's Hospital at Westmead, Westmead, New South Wales, Australia.
J Clin Neurosci. 2013 Apr;20(4):514-9. doi: 10.1016/j.jocn.2012.04.017. Epub 2013 Jan 11.
A retrospective analysis was conducted on consecutive patients with intracranial germ cell tumours diagnosed and treated from 1 January 1997 to 31 December 2007 to assess and determine demographic factors and treatment outcomes of children with these tumours treated in a major paediatric referral hospital in Australia. In this study, intracranial germ cell tumours represented 4.8% of paediatric brain tumours seen. Of the 21 patients identified, 15 (71.4%) were diagnosed with pure germinoma and six (28.6%) with non-germinomatous germ cell tumours (NGGCT) or mixed tumours. One patient received chemotherapy alone, two patients were treated with radiation alone and the remaining 18 received a combination of chemotherapy and radiotherapy. A total of 33 neurosurgical operations were performed with 15 biopsies via open, endoscopic or transphenoidal means; nine open resections; and nine procedures for hydrocephalus comprising seven third ventriculostomies and two ventriculoperitoneal shunts. For patients with pure germinomas, the 5-year disease-free rate (DFS) was 93.3%, and overall survival (OS) rate was 100% compared to NGGCT or mixed tumours (DFS 50%; OS 50%) (DFS p=0.019, OS p=0.004). The data presented show that pure germinomas carry a favourable prognosis. The data also support that treatment with induction chemotherapy followed by dose-attenuated radiotherapy is an effective alternative with results comparable to historical controls treated with craniospinal irradiation. Although chemoradiotherapy has become the mainstay of treatment in intracranial germ cell tumours, surgery remains integral to the management of this condition. Surgery remains important in establishing the histological diagnosis, as well as in the treatment of hydrocephalus. Furthermore, debulking procedures may be advocated in NGGCT as they are often resistant to chemotherapy.
对 1997 年 1 月 1 日至 2007 年 12 月 31 日连续诊断和治疗的颅内生殖细胞瘤患者进行回顾性分析，以评估和确定在澳大利亚主要儿科转诊医院治疗的这些肿瘤患儿的人口统计学因素和治疗结果。在这项研究中，颅内生殖细胞瘤占儿科脑肿瘤的 4.8%。在确定的 21 名患者中，15 名（71.4%）被诊断为单纯生殖细胞瘤，6 名（28.6%）为非生殖细胞瘤生殖细胞瘤（NGGCT）或混合瘤。一名患者单独接受化疗，两名患者单独接受放疗，其余 18 名患者接受化疗和放疗联合治疗。共进行了 33 次神经外科手术，其中 15 次通过开颅、内镜或经蝶窦方法进行活检；9 次开颅切除术；9 次脑积水手术，包括 7 次第三脑室造口术和 2 次脑室-腹腔分流术。对于单纯生殖细胞瘤患者，5 年无疾病生存率（DFS）为 93.3%，总生存率（OS）为 100%，而非生殖细胞瘤生殖细胞瘤或混合瘤（DFS 为 50%；OS 为 50%）（DFS p=0.019，OS p=0.004）。所呈现的数据表明，单纯生殖细胞瘤具有良好的预后。这些数据还支持，与接受颅脊髓照射治疗的历史对照相比，采用诱导化疗后进行剂量降低放疗是一种有效的替代方法。虽然化学放疗已成为颅内生殖细胞瘤治疗的主要方法，但手术仍然是该疾病治疗的重要组成部分。手术对于建立组织学诊断仍然很重要，并且对于治疗脑积水也很重要。此外，在 NGGCT 中提倡进行减瘤手术，因为它们通常对化疗有抵抗力。
