Yecies Derek, Fisher Paul Graham, Cheshier Samuel, Edwards Michael, Grant Gerald
1Division of Pediatric Neurosurgery, Department of Neurosurgery, and.
2Division of Child Neurology, Department of Neurology, Stanford University School of Medicine, and Lucile Packard Children's Hospital Stanford, California.
J Neurosurg Pediatr. 2018 Jan;21(1):49-53. doi: 10.3171/2017.7.PEDS17168. Epub 2017 Nov 10.
OBJECTIVE Primarily metastatic juvenile pilocytic astrocytoma (JPA) is rare, likely representing 2%-3% of all cases of JPA. Due to the rarity of primarily metastatic JPA, there is currently no standard treatment paradigm and the long-term outcomes are not fully known. The goal of this case series was to add to the current understanding of this disease process. METHODS The authors searched a comprehensive database of pediatric patients with brain and spinal cord tumors treated at Lucile Packard Children's Hospital from 1997 to 2016 and identified 5 patients with primarily metastatic JPA. A retrospective chart review was performed and details of the patients' treatment and clinical course were recorded for further analysis. RESULTS For the 5 patients with primarily metastatic JPA, the mean follow-up period was 12.3 years. All patients in our series had biopsies or subtotal resections and upfront treatment. Three patients were treated with chemotherapy alone, one was treated with chemotherapy and radiotherapy, and one was treated with radiotherapy alone. Four patients had stable disease after initial treatment, and one patient had multiple episodes of progressive disease but underwent successful salvage therapy and has had stable disease for 19 years. One patient died of an intracerebral hemorrhage 10 years following initial radiation treatment believed to be secondary to radiation vasculopathy. CONCLUSIONS Evaluation of the entire neuraxis should be performed in all instances of initial JPA diagnosis to properly assess for primarily metastatic disease. Many patients with primarily metastatic JPA will have stable disease after upfront treatment, although the higher rate of stable disease found in this series relative to other reports is likely secondary to the small sample size.
目的 原发性转移性青少年毛细胞型星形细胞瘤（JPA）较为罕见，可能占所有JPA病例的2%-3%。由于原发性转移性JPA罕见，目前尚无标准治疗模式，长期预后也不完全清楚。本病例系列的目的是增进对这一疾病过程的现有认识。方法 作者检索了1个综合数据库，该数据库涵盖1997年至2016年在露西尔·帕卡德儿童医院接受治疗的患有脑和脊髓肿瘤的儿科患者，共识别出5例原发性转移性JPA患者。进行了回顾性病历审查，并记录了患者的治疗和临床过程细节以供进一步分析。结果 对于5例原发性转移性JPA患者，平均随访期为12.3年。我们系列中的所有患者均接受了活检或次全切除及初始治疗。3例患者仅接受化疗，1例接受化疗和放疗，1例仅接受放疗。4例患者初始治疗后病情稳定，1例患者有多次疾病进展，但接受了成功的挽救治疗，病情稳定19年。1例患者在初始放疗10年后死于脑出血，据信是由放射性血管病继发所致。结论 在所有初始诊断为JPA的病例中均应进行全神经轴评估，以正确评估原发性转移性疾病。许多原发性转移性JPA患者在初始治疗后病情会稳定，尽管本系列中发现的稳定疾病发生率高于其他报告，这可能是由于样本量小所致。
