A Retrospective Analysis of Recurrent Intracranial Ependymoma
A Retrospective Analysis of Recurrent Intracranial Ependymoma
复制标题
DOI：
10.1002/pbc.24996
复制
发表时间：
2014-07-01
影响因子：
3.2
通讯作者：
Finlay, Jonathan L.
中科院分区：
文献类型：
--
作者：
Antony, Reuben;Wong, Kenneth E.;Finlay, Jonathan L.
背景 患有髓母细胞瘤的儿童有多种初始症状。然而，潜在的肿瘤生物学对髓母细胞瘤最初表现的影响目前尚不清楚。鉴于最近发现了不同的髓母细胞瘤亚组，我们试图以亚组特定的方式定义儿童髓母细胞瘤的初始表现。程序 我们收集了 1994 年至 2012 年间病童医院的 126 例髓母细胞瘤病例，并使用 nanoString 确定了亚组隶属关系。与初始表现有关的临床细节是通过回顾性图表审查确定的。结果 所有髓母细胞瘤病例的中位诊断前间隔为 4 周（IQR：4-12 周）。引人注目的是，当以亚组特定方式确定诊断前间隔时，WNT 和第 4 组肿瘤病例的中位诊断前间隔为 8 周，而 SHH 为 2 周，第 3 组为 4 周（P = 0.0001）。年龄较小与诊断前间隔延长显着相关（所有 P = 0.02）。当按亚组分层时，与年龄的相关性仅在第 4 组中显着（第 4 组 P = 0.04）。生存率的提高与较长的诊断前间隔显着相关（P = 0.02），但在控制亚组时不再显着（P = 0.07）。结论 儿童髓母细胞瘤诊断前间隔的持续时间高度依赖于亚组，进一步凸显了髓母细胞瘤四个主要亚组的临床异质性和生物学相关性。儿科血癌 2014 年；61：1190-1194。 (c) 2014 年 Wiley 期刊公司。
Background Children presenting with medulloblastoma have a wide range of initial presenting symptoms. However, the influence of underlying tumor biology on the initial presentation of medulloblastoma is currently unknown. In light of the recent discovery of distinct medulloblastoma subgroups, we sought to define the initial presentation of childhood medulloblastoma in a subgroup specific manner. Procedure We assembled a cohort of 126 medulloblastoma cases at the Hospital for Sick Children between 1994 and 2012 and determined subgroup affiliation using nanoString. Clinical details pertaining to the initial presentation were determined through a retrospective chart review. Results The median pre-diagnostic interval across all medulloblastoma cases was 4 weeks (IQR: 4-12 weeks). Strikingly, when the pre-diagnostic interval was then determined in a subgroup specific manner, cases with WNT and Group 4 tumors showed significantly longer median pre-diagnostic intervals of 8 weeks compared to 2 weeks for SHH and 4 weeks for Group 3 (P = 0.0001). Younger age was significantly associated with a prolonged pre-diagnostic interval (P = 0.02 for all). When stratifying by subgroup the association with age was only significant in Group 4 (P = 0.04 for Group 4). Improved survival was significantly associated with a longer pre-diagnostic interval (P = 0.02), however is no longer significant when controlling for subgroup (P = 0.07). Conclusions The duration of the pre-diagnostic interval in childhood medulloblastoma is highly subgroup dependent, further highlighting the clinical heterogeneity and biological relevance of the four principle subgroups of medulloblastoma. Pediatr Blood Cancer 2014;61:1190-1194. (c) 2014 Wiley Periodicals, Inc.