Ronsley Rebecca, Bouffet Eric, Dirks Peter, Drake James, Kulkarni Abhaya, Bartels Ute
1Division of Hematology, Oncology & Bone Marrow Transplant, Department of Pediatrics, British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia.
2Division of Hematology, Oncology & Bone Marrow Transplant, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Ontario; and.
J Neurosurg. 2021 Dec 3;137(3):807-812. doi: 10.3171/2021.8.JNS211443. Print 2022 Sep 1.
The objective of this study was to describe the management of hydrocephalus in a cohort of pediatric patients with germinoma.
The authors conducted a retrospective chart review of patients with germinoma and symptomatic hydrocephalus treated at the Hospital for Sick Children between 2002 and 2020. Descriptive data included tumor location, CSF diversion procedure (external ventricular drain [EVD], endoscopic third ventriculostomy [ETV], ventriculoperitoneal [VP] shunt) and outcomes. The frontooccipital horn ratio (FOR) method was used to determine the presence of ventriculomegaly.
Of 39 patients with germinoma, 22 (73% male) had symptomatic hydrocephalus at diagnosis (11 pineal, 4 suprasellar, and 7 bifocal). Management of hydrocephalus included EVD (n = 5, 22.7%), ETV (n = 5, 22.7%), and combination ETV and EVD (n = 7, 31.8%), whereas 5 patients (22.7%) did not undergo surgical intervention. The median FOR at diagnosis was 0.42 (range 0.38-0.58), which correlated with moderate to severe ventriculomegaly. Carboplatin and etoposide-based chemotherapy induced fast tumor shrinkage, avoiding CSF diversion (n = 5) and resolving hydrocephalus with a transient EVD (n = 5). The median duration until EVD removal was 7 days (range 2-10 days). Two of 12 patients with EVD ultimately required a VP shunt. Kaplan-Meier overall survival was 100% and progression-free survival was 96.4% at a median follow-up of 10.4 years.
Timely initiation of chemotherapy is imperative to rapidly reduce tumor bulk in children with germinoma and limits the need for VP shunt insertions. In children in whom CSF diversion is required, hydrocephalus may be successfully managed with a temporary EVD ± ETV.
本研究的目的是描述一组患生殖细胞瘤的儿科患者中脑积水的治疗情况。
作者对2002年至2020年在病童医院接受治疗的患有生殖细胞瘤和症状性脑积水的患者进行了回顾性病历审查。描述性数据包括肿瘤位置、脑脊液分流手术（外部脑室引流[EVD]、内镜下第三脑室造瘘术[ETV]、脑室腹腔[VP]分流术）及结果。采用额枕角比率（FOR）法确定脑室扩大的情况。
39例生殖细胞瘤患者中，22例（73%为男性）在诊断时患有症状性脑积水（11例松果体区、4例鞍上区和7例双灶性）。脑积水的治疗包括EVD（n = 5，22.7%）、ETV（n = 5，22.7%）以及ETV和EVD联合治疗（n = 7，31.8%），而5例患者（22.7%）未接受手术干预。诊断时的中位FOR为0.42（范围0.38 - 0.58），这与中度至重度脑室扩大相关。基于卡铂和依托泊苷的化疗导致肿瘤快速缩小，避免了脑脊液分流（n = 5），并通过临时EVD解决了脑积水问题（n = 5）。EVD拔除前的中位持续时间为7天（范围2 - 10天）。12例接受EVD治疗的患者中有2例最终需要VP分流术。在中位随访10.4年时，Kaplan-Meier总生存率为100%，无进展生存率为96.4%。
对于患生殖细胞瘤的儿童，及时开始化疗对于迅速缩小肿瘤体积至关重要，并限制了VP分流术的插入需求。对于需要脑脊液分流的儿童，脑积水可通过临时EVD ± ETV成功治疗。
